I was just Diagnosed with Raynaud's a few days ago by my Rheumy. I have a lot of blood work to get done, and since I do not have insurance, I am having to split the test up in groups( talk about expensive on self pay).I am awaiting my ana, hep panel,cbc,cmp,cpk,and rf to come in.
Anywho, since I have about a month til my 1st round of results come in, I just wanted an opinion on if it's likely that I have a connective tissue dissorder, and If my Raynauds sounds like a severe case. Here is a somewhat brief description of what I have going on.
I had seen my GP before the rheumy, and my hands, and feet were swollen, burned, ached,and had stabbing pains. I had flesh colored bumps under my toes, purple bruises, and red bumps that quickly turned into dime sized ulcers on the top of my toes. I did notice that my toes were bone chilling cold at times, so the raynauds makes since.However, with all of the other pain i was in, it was the last thing on my mind. My GP thought I had a form of arthritis.
My rheumy confirmed i do not , and all joints look good. After seeing my GP I started having severe sensitivity to light. Lots of floaters when in daylight or any bright light, blurry vision, headaches, and flashes of purple light everytime I drove at night. After that I started having sharp pains all over my body. So painful that it brought me to tears. I would feel hot one minute and the next have chills, and felt dizzy. I then started having episodes of intense chest pains, rapid heart beat with some skipped beats,and shortness of breath. After a few attacks, I was actually sleeping and awoken from my sleep by irregular heat beat, pain, and dizzines. That is actually what made me break down and go see a rheumy.
Other than the Raynauds, she said it could be nothing or possibly a connective tissue disorder. She said that since I did not have a rash, she though it was unlikely to have lupus.
1. Does my raynauds sound like primary, or secondary?
2. Do my symptoms sound like I may have something else going on with my Raynauds, or is it typical Raynauds symptoms?
Was a nailfold capillaroscopy done? That can help provide some helpful information. You didn't say if your ulcers healed quickly and for how long you've had Raynaud's. You also don't mention how young you are. So many things can cause your symptoms, both related to and unrelated to AI problems. It is very good that you don't have joint damage. I hope that you keep us updated on your labs and are getting treatment for your Raynaud's to prevent further ulceration!
I am 29, and no nailfold capillaroscopy has been done. Unfortunatley the ulcers have not healed, I noticed them almost 2 months ago, and since then, new ones have appeared. I am not sure as to how long I have had Raynauds. It was just diagnosed last week. Since, I have posted my original thread, I have been having sick spells where i get very dizzy, sick feelings, chills, and shaky vision ( only during the sick spells). Any insight would be great. A month is an awful long time to live with these symptoms, with no clue on what's going on with my body. Thank you
Did your Dr give you anything for the ulcers? And any antibiotics in case you got an infection that goes systemic? You must take care of those ulcers lest your circulation gets compromised. Does a thick, chalky white residue that is not pus ooze or come out of your ulcers? That symptom can be indicative of an autoimmune problem but not definately. Same if you have little red dots, or what is sometimes called spider veins. Those sharp pains could be associated w/ Raynaud's as neuropathy but I'm not a Dr, just a patient. I have found that simple anti inflammatory OTC medications to be the most helpful for my pains, which Are from secondary Raynaud's. As for the fevers and chills, keep warm and try to reduce your stress. Yeah right, you are probably thinking, but it could be that you just have a virus or something like that. Unfortunately, your Dr is right, you may or may not have an AI disease at this point. And if you do have something besides Raynaud's, stress reduction is a key part of treatment.
Last edited by luca689; 03-05-2012 at 02:09 PM.
Reason: forgot something
Primary is much more common but ulcers do not usually occur with primary so secondary would be the other option there are many many other CTDs out there than lupus. I have raynauds with an underlying CTD I am still in the process of finding out which one. Mine is probably either lupus or scleroderma. I am only 26 and sometimes these diseases take time to progress. So unless you had a really bad month or two if the health issues persist I would keep perusing it! there are nail fold capillary changes that a doctor can look at with there scope and primary raynauds looks normal an abnormal result would point at a CTD but which one is the question that is the point that I am at good luck!
NinNin, It's good to know that I am not the only one experiencing this. The whole process seems to be never ending. What symptoms were you having when diagnosed with Raynaud's? You said that you suspect scleroderma, have you noticed any thickening of skin, on your legs, hands or face? I'm just curious to know if you have experienced any of the same symptoms that I have.
Gdiluca, My Dr. did not give me anything for the Ulcers, and no antibiotics. There is nothing oozing out of them. They are a redish purple color, and about the size of a dime. The only thing that my Dr. put me on was 25 mg Lyrica for pain so that I could actually sleep at night ( which i stopped taking cause it made me feel worse). She told me to keep my feet elevated when possible, and to wear socks to bed.The dr. wanted to get my test results back before going any further. You asked me about little red dots... Could you describe what type of red dots? I do have a few red dots on my feet, and underneath them are small hard bumps ( feels like bone, or calcium deposits). I also have needle sized red dots on my hands , and arms that are smooth, with no bumps underneath. Thanks for the reply.
I went in to see a doctor becaues the skin under my nails were blue and within a few days of that my whole toes were turning blue. At the time I had been diagnoesd with Still's Disease so raynauds was kinda thrown at me from out at left field that was a year ago. Because I had raynauds they started checking for scleroderma and I soon learned that I had many of the same issues as CREST raynauds esophageal issues and the telangiectasia. i dont have skin thickening or didn't I am getting some around the fingernails I'm not so sure it's all scleroderma related or not. As it would be an atypical presentation. You really need to get on some type of cream there is no need for blood tests to treat the raynauds. The ulcers would almost certainly point at a CTD IMO. I've learned a lot about this if you have any qustions, just ask.
Lyrica! omg there are plenty of meds to help with blood circulation unless you have heart issues they are usually BP meds and can drop you BP.. i was on those for a year there is also nitro-bid paste and revatio that works really well but you have to get a doctor to approve it!
My dr. did say that if my symptoms did not improve in a month that she was going to put me on blood pressure meds to help with circulation in my hands and feet. What would the cream that you are speaking of help? I only took the lyrica one time and it made me feel awful and almost seemed to make my symptoms worse. She is not doing blood test for the Raynaud's. She is doing all of the blood test to see if I have another disease linked with the Raynaud's. Not to mention that some of the symptoms that I am having sound seriuos, and are really stressing me out( The chest pains, dizzy, and sick spells, and the fact that my vision has been affected). I actually had these issues about 10 years ago but without the Raynaud's. They had tested for lupus and done MRI'S and could never figure out what was causing any of it. Since that was ten years ago, she wants to redo a lot of the test since lupus can present at any time. I am leaning towards scleroderma as one of my ctd's. The skin on my right hand has become so thick over the past two months that I can no longer wear any of my rings that still fit my left hand. I have tried all kinds of anti inflammatories to see if the skin thickening was caused by inflammation and nothing will help. I have a few more weeks until my test come in, and I go back to see the doctor.
The following user gives a hug of support to nicoletx: luca689 (03-09-2012)
Well I do not get sores. I have slow heal time when I injure myself If I get a blister it stay forever. I get cold numbed and stinging fingers. Even when they are cold they can burn. I get lots of dents in my nails (not sure from what exactly) but i have really bad nail fold capillaries the capillaries bleed out into my cuticles so i have red spotted cuticles sometimes they bleed so much it comes up from underneath the cuticle where I can't actually wash the blood off. It doesn't happen very often, and certainly doesn't happen with primary raynauds! My feet and hands are effected and I hurt myself pretty easily b/c they are so numb but other than cuts and bruises no ulcers to date! my feet get bluer than my hands but my hands are much more painful and sometiems they swell up in the knuckles but are still cold to the touch go figure...lol i get the bleedson my toes as welll. And get quite a few splinter hemmorhages at a time when i do get them but i don't have them all of the time.
The following user gives a hug of support to NinNin: luca689 (03-09-2012)
The hard lumps I have seen described by several other people as being diagnosed as calcinosis. However, I am not a Dr but only a patient and only your Dr can diagnose you!. Primary Raynaud's Can exhibit capillary dropout if the vascular damage is extreme or has occurred over a long period of time, so that is why a nailfold test is not completely definitive. That is why I asked your age. Skin thickening of the hands in scleroderma usually follows a bilateral pattern of swollen puffy fingers and/or toes so much so that the look like sausages and then comes the skin hardening, in limited systemic scleroderma. There is also a form of scleroderma called sine in which the skin never thickens or hardens. Let's see, I wanted to address the medications for Raynaud's. They are usually calcium channel blockers to start, and although not everyone benefits from them they certainly help most people. I've been on several throughout the years. The chest pains may very well be due to stomach and esophageal problems, as it was in my case. BUT, again, only your Dr can tell for sure. I also wanted to mention that ocular migraines can cause vision problems and can be caused by scleroderma. As far as your ulcers, you really need to address those. Many people use loose gauze, splints on the non ulcer sides of their fingers to keep from knocking them about, which will help with the pain somewhat. I've heard of many people using various types of OTC antibiotics as well. People have also had success with nitroglycerine Rx creams and Ravatio Rx creams. Staying warm is crucial as well. I always have gloves with me, even in the middle of summer, LOL, especially if I have to go to the grocery store, or even drinking a cold glass of lemonade! As you have probably already guessed by now, I have one of the forms of scleroderma. It is a rather rare disease that many Dr's are unfamiliar with. If anyone has any specific questions or just want some support, please feel to PM me. I've had this diagnosis for, geez, almost 10 yrs.
Last edited by luca689; 03-09-2012 at 10:43 AM.
Reason: clarification
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Gdiluca - I have done a lot of research on nail fold capillaries and I am wondering where you learned some of this info from you seem very knowledgeable! Usually the NFC test is the gold standard in differentiating between Primary and Secondary Raynauds and not everyone with a CTD will have abnormal capillaries but yes if there is damage to the nail fold area it can change it but there are very distinct patterns for each individual disease. This is where it really intrigues me Lupus, Scleroderma, Dermamyositis, APS, and UCTD all have very distint patterns. There is a Dr named Dr. Cutolo who is working on making it a diagnostic criteria for Scleroderma over in the UK! I hope the US gets to this point as well. I am a total science geek and got a USB microscope they cost like $24 and took my own pictures since I was told they were abnormal but then the doctor that said that left her practice and I am having trouble getting anyone else to look at it. I had a doctor go as far as to tell me that my capillaries are my "normal" nothing I've read says this is a normal phenomena but anyways. I took pictures I haven't been on this site in a while I'll have to see if they do photo sharing still. the original rheumy said it was scleroderma pattern but my other doctors haven't looked and I don't know what it all means i wish a doctor could tell me..lol
The cream is called Nitro-Bid paste and it's the only medicine that I know of that isn't ingested for Raynauds and you have to be really careful for it because it is nitroglycerin cream that people use for chest pain but you use it on your hands and feet and it helps dialate the capillaries
From my understanding of calcinosis you can get a simple xray and if it is a calcium deposit it should light up like a light bulb on the film! But they will still want to run all of those blood tests anyways it may be time to consider getting a good insurance!
Are you sure that you are not talking about the ANA patterns, rather than the nailfold capillaries. That is called "drop out" and is caused by vascular damage regardless of cause, be it age, long term damage, or scleroderma. This is not so much really a Gold Standard but it is highly indicative given the history of any one individual with Raynaud's and is, to my knowledge, only done in context with the presence of Raynaud's, The ANA patterns are indeed different most of the time with the various AI diseases, but not necessarily so. For instance, the anticentromere pattern almost, note the almost part, occurs with limited systemic scleroderma. But it also occurs in lupus patients as well as PBC patients. And to make things even more confusing, many patients with limited SSc do not have a positive ANA at all. I've had scleroderma for a while now, and have time to talk to other patients, my Dr's, and have had time to research a few things. My sources include accredited, peer reviewed rheumatology journals and the NIH, CDC, and such.
X-rays can be used for detecting calcinosis but is expensive and a specialist can usually determine if it's calcinosis without the expense and exposure to x-rays.
No there are patterns in the nail fold capillascopy I've done a lot of reseach on it.. each disease has it's own pattern lupus makes them long an narrow aps causes bleeds but normal looking capillaries DM causes bleeds and big twisted capillaries with drop outs uhm and both types of scleroderma have their own unique patterns. It's very hard to find unless you know what you are looking for .. but there are patterns
Dr. Cutolo wrote this he is the doctor that i spoke of he is head of the research in the UK
Nailfold capillaroscopy is useful for the diagnosis and follow-up of autoimmune rheumatic diseases. A future tool for the analysis of microvascular heart involvement?
M. Cutolo, A. Sulli, M. E. Secchi, S. Paolino and C. Pizzorni
+ Author Affiliations
Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy.
Correspondence to: Maurizio Cutolo, Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy. E-mail: mcutolo@unige.it
Next Section
Abstract
Raynaud's phenomenon (RP) represents the most frequent clinical aspect of cardio/microvascular involvement and is a key feature of several autoimmune rheumatic diseases. Moreover, RP is associated in a statistically significant manner with many coronary diseases. In normal conditions or in primary RP (excluding during the cold-exposure test), the normal nailfold capillaroscopic pattern shows a regular disposition of the capillary loops along with the nailbed. On the contrary, in subjects suffering from secondary RP, one or more alterations of the capillaroscopic findings should alert the physician of the possibility of a connective tissue disease not yet detected. Nailfold capillaroscopy (NV) represents the best method to analyse microvascular abnormalities in autoimmune rheumatic diseases. Architectural disorganization, giant capillaries, haemorrhages, loss of capillaries, angiogenesis and avascular areas characterize >95% of patients with overt scleroderma (SSc). The term ‘SSc pattern’ includes, all together, these sequential capillaroscopic changes typical to the microvascular involvement in SSc. The capillaroscopic aspects observed in dermatomyositis and in the undifferentiated connective tissue disease are generally reported as ‘SSc-like pattern’. Effectively, and early in the disease, the peripheral microangiopathy may be well recognized and studied by nailfold capillaroscopy, or better with nailfold video capillaroscopy (NVC). The early differential diagnosis between primary and secondary RP is the best advantage NVC may offer. In addition, interesting capillaroscopic changes have been observed in systemic lupus erythematosus, anti-phospholipid syndrome and Sjogren's syndrome. Further epidemiological and clinical studies are needed to better standardize the NCV patterns. In future, the evaluation of nailfold capillaroscopy in autoimmune rheumatic diseases might represent a tool for the prediction of microvascular heart involvement by considering the systemic microvascular derangement at the capillary nailfold.
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Introduction
The story of capillaroscopy ‘started’ from the observations of an Italian physician, Giovanni Rasori (1766–1837), who described the close relationship between conjunctival inflammation and the presence of an ‘inextricable knot of capillary loops’ by a magnifying glass [1].
During the beginning of the 20th century, Brown and O'Leary [2] used the capillaroscopic analysis to show in detail the abnormalities that characterize the involvement of microvasculature during Raynaud's phenomenon (RP) in systemic sclerosis (Ssc). In 1973, Maricq et al. [3] published the first article in Arthritis and Rheumatism, describing the specific capillaroscopic patterns in Ssc as well as the modification of the capillary blood flow during cold exposure, both in primary and secondary RP [3]. Nailfold videocapillaroscopy (NVC) represents the best method to analyse micro/cardiovascular abnormalities in rheumatic diseases. In normal conditions, the microvascular pattern is characterized by a regular array of microvessels with large intra/interindividual variability. However, absolute absence of capillary loss and giant capillaries is expected in normal pattern.
Recently, three defined major NVC patterns have been considered useful in assessing the appearance and progression of the sclerodermic microangiopathy (‘early’, ‘active’ and ‘late’ patterns) [4].
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The ‘scleroderma pattern’
The microvascular damage in SSc is mainly characterized by increasing structural alterations of the capillaries with progressive decrease in their density [5, 6]. Blood flow is also altered, with an average slowing of flow and increased periods of stasis.
Early in the disease, the peripheral microangiopathy may be well recognized and studied by nailfold capillaroscopy or better with the videocapillaroscopy, a non-invasive and safe technique, which is well reported to have both diagnostic and prognostic values in the presence of RP [6–11].
Previous studies have partially graded the morphological aspects of the vascular damage in patients with SSc, as assessed by nailfold capillaroscopy, and two major patterns within the term ‘scleroderma pattern’ (SSc pattern) have been recognized from the beginning: namely the ‘active’ and the ‘slow’ patterns [7].
Further morphological studies have been published more recently [12, 13].
In a recent study, microvascular alterations as detected by NVC in patients with SSc have been re-classified in three different patterns [4].
The patterns identified within the ‘SSc pattern’ include: (i) ‘Early’ NVC pattern: few enlarged/giant capillaries, few capillary haemorrhages, relatively well-preserved capillary distribution, no evident loss of capillaries (Fig. 1); (ii) ‘Active’ NVC pattern: frequent giant capillaries, frequent capillary haemorrhages, moderate loss of capillaries, mild disorganization of the capillary architecture, absent or mild ramified capillaries (Fig. 2A); and (iii) ‘Late’ NVC pattern: irregular enlargement of the capillaries, few or absent giant capillaries and haemorrhages, severe loss of capillaries with extensive avascular areas, disorganization of the normal capillary array, ramified/bushy capillaries (Fig. 1).
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FIG. 1.
A, early SSc pattern; B, active SSc pattern; C, late SSc pattern and D, normal pattern (magnification 200×).
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FIG. 2.
A, active SSc pattern (magnification 200×); B, pattern observable in patients with UCTD (magnification 200×); C, with DM (magnification 200×) and D, with APS (magnification 200×).
This study confirmed previous observations, indicating enlarged and giant capillaries together with haemorrhages as the earliest NVC finding in SSc. In the late stage of the disease, these abnormalities become rare.
Early stage is also characterized by microvessels with normal diameter coexisting with few enlarged capillaries that must be carefully investigated on all the fingers by considering the limited number of these nailfold changes during early phases of the disease [14–16].
Conversely, these changes are strongly increased in SSc patients with an ‘active’ pattern. Loss of capillaries, together with vascular architectural disorganization and ramified capillaries, were found to be rare in the early stages of SSc, whereas they seem to increase with the progression of the fibrotic phase of the disease (‘active’ and ‘late’ patterns).
A significant and gradual increase of these latter vascular abnormalities is observed during the SSc progression, and the three NVC patterns have been found to correlate with both RP and SSc durations, reflecting at least the possible evolution of the disease process [4].
While in healthy control subjects and in patients with primary RP the morphological features of the nailfold microvascular bed may remain unchanged for a long time, patients with RP associated with SSc-spectrum disorders may, on the contrary, show a higher degree of morphological variability, even after a few days.
The NVC patterns have been correlated with different clinical aspects and manifestations of SSc, as well as to the effects of treatment contributing to the overall study of the disease [17–21].
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Capillaroscopic patterns and rheumatic diseases
The presence of megacapillaries and a decreased capillary density are the hallmarks of the SSc capillary pattern, which can be detected by nailfold capillaromicroscopy.
However, in a recent large study, 186 patients with RP, 65 with undifferentiated connective tissue disease (UCTD), 47 with systemic lupus erythematosus (SLE), 26 with dermato/polymyositis, 14 with rheumatoid arthritis, 7 with primary Sjogren's syndrome (SS) and 102 patients with SSc were investigated [22].
Of the 16 patients with diffuse cutaneous SSc and the 86 with limited cutaneous SSc, 14 (87.5%) and 53 (61.6%), respectively, showed the SSc capillary pattern.
Nine of the 65 (13.8%) cases with UCTD and 24 of the 186 (12.9%) cases with RP also exhibited the same pattern.
Four of the 47 (8.5%) patients with SLE and seven of the 26 (26.9%) with dermato/polymyositis, and no patients with rheumatoid arthritis or SS exhibited the SSc capillary pattern.
The conclusion is that the ‘SSc pattern’ is often present in SSc and dermato/polymyositis. Furthermore, patients with RP and UCTD may also exhibit this pattern occasionally (Fig. 2B). Therefore, capillaromicroscopy seems to be a useful tool for the early selection of those patients who are potential candidates for developing SSc spectrum disorders.
Dermatomyositis
A defined pattern has been reported in patients affected by dermatomyositis (DM) [23]. This pattern, often associated with aspects of the SSc pattern, includes the presence of two or more of the following findings in at least two nail folds: enlargement of capillary loops, loss of capillaries, disorganization of the normal distribution of capillaries, ‘budding’ (‘bushy’) capillaries, twisted enlarged capillaries and capillary haemorrhages (extravasates) (Fig. 2C) [24].
Systemic lupus erythematosus
Characteristic SLE pattern includes morphological alterations of capillary loops, venular visibility and sludging of blood with variability of capillary loop length [25]. A recent study evaluated the association between nailfold capillary abnormalities frequently observed in SLE patients and the presence of RP, anti-U1-ribonucleoproteins (RNP) and anti-cardiolipin (aCL) antibodies [26].
One hundred SLE patients were studied. The widefield nailfold capillaroscopy was considered abnormal according to five criteria. Intercapillary distance, capillary width and capillary length were registered by videomorphometry in two fingers in all the 100 patients and in four fingers in 40 of them. Both the presence of altered capillaroscopy and that of the SSc pattern, characterized by the presence of avascular areas and enlarged or giant loops, were associated with the isolated presence of RP (P < 0.001) or anti-U1-RNP antibodies (P < 0.01), as well as with the simultaneous presence of RP and anti-U1-RNP antibodies (P < 0.001). There was a negative association between the presence of aCL antibodies and SSc pattern (P < 0.05). Higher figures for the videomorphometric parameters capillary width, intercapillary distance and capillary length were observed in patients with RP. Patients presenting both RP and anti-U1-RNP antibodies showed higher figures for intercapillary distance and capillary width. This study demonstrated significant association between nailfold capillaroscopic abnormalities and either RP or anti-U1-RNP antibodies in SLE patients. The association of RP, anti-U1-RNP antibodies and ‘SSc like’ findings on nailfold capillaroscopy (SSc pattern) in patients with SLE may suggest a new SLE subset with subclinical features of SSc.
A previous study evaluated the relationship between aCL (found in about 40–50% of patients suffering from SLE) and skin microcirculatory changes or vascular symptoms in 51 consecutive SLE patients [27].
Twenty-two patients (43.1%) had positive aCL [immunoglobins G (IgG) 22 (5–60) G phospholipids (GPL); immunoglobins M (IgM) 5 (3–16.5) M phospholipids (MPL); median titre and range, respectively] and 12 (54.5%) of them had abnormal capillaroscopic findings. In contrast, among the 29 patients without aCL, only six (20.7%) had an abnormal capillaroscopy (P = 0.027). There was no correlation between either aCL or capillaroscopy and RP. These results showed a relationship between aCL and nailfold capillary changes in patients with SLE, suggesting a direct damage of the vascular endothelium by aCL.
Anti-phospholipid syndrome
As a matter of fact, interesting microvascular alterations have been observed in patients affected by anti-phospholipid syndrome (APS). One study reported symmetrical microhaemorrhages at nailfold analysis, which were found particularly significant in patients showing the presence of both serum IgG and IgM ACL (Fig. 2D) [28]. Marked microcirculatory damage was found in relation with the occurrence of thrombotic manifestations in the APS patients in other studies, confirming the pattern [27].
A more recent study confirmed that nailfold capillary morphology is altered in patients with APS, but these changes could not be correlated to the impairment of functional parameters [29].
Sjogren's syndrome
Capillaroscopic changes have been observed also in primary SS [30]. Forty patients with SS (14 without RP, 16 with RP, 10 with ACA), 20 patients with SSc (10 with limited and 10 with diffuse disease) (disease control group) and 40 healthy controls (control group) were evaluated by nailfold capillaroscopy. Capillaroscopic abnormalities in SS ranged from non-specific findings (crossed capillaries) to more specific findings (confluent haemorrhages and pericapillary haemorrhages) or SSc-type findings. SS patients with RP presented capillary abnormalities in higher frequency than patients without RP. The majority of SS patients with ACA (80%) presented SSc-type findings. Nailfold capillaroscopy can be used as a simple non-invasive method to evaluate the microvascular abnormalities in SS patients, especially in those with RP and with ACA [31].
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Conclusions
Since in a recent study, duplex determination of the carotid elasticity or stiffness was found significantly impaired in secondary RP associated with SSc when compared with primary RP, the early capillaroscopic differential diagnosis between the two forms might improve the cardiac prognosis [32].
In fact, the recognition of the capillaroscopic patterns characterizing the secondary RP might be prognostic even for the early detection of the risk of visceral RP (i.e. coronary heart disease) [33].
In conclusion, the regular evaluation of nailfold capillaroscopy in autoimmune rheumatic diseases will represent soon a tool for the analysis even of the microvascular heart involvement by considering the systemic involvement of the microvessels in these diseases [34, 35].
Dr. Cutolo wrote this he is the doctor that i spoke of he is head of the research in the UK
Nailfold capillaroscopy is useful for the diagnosis and follow-up of autoimmune rheumatic diseases. A future tool for the analysis of microvascular heart involvement?
M. Cutolo, A. Sulli, M. E. Secchi, S. Paolino and C. Pizzorni
+ Author Affiliations
Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy.
Correspondence to: Maurizio Cutolo, Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy. E-mail: mcutolo@unige.it
Next Section
Abstract
Raynaud's phenomenon (RP) represents the most frequent clinical aspect of cardio/microvascular involvement and is a key feature of several autoimmune rheumatic diseases. Moreover, RP is associated in a statistically significant manner with many coronary diseases. In normal conditions or in primary RP (excluding during the cold-exposure test), the normal nailfold capillaroscopic pattern shows a regular disposition of the capillary loops along with the nailbed. On the contrary, in subjects suffering from secondary RP, one or more alterations of the capillaroscopic findings should alert the physician of the possibility of a connective tissue disease not yet detected. Nailfold capillaroscopy (NV) represents the best method to analyse microvascular abnormalities in autoimmune rheumatic diseases. Architectural disorganization, giant capillaries, haemorrhages, loss of capillaries, angiogenesis and avascular areas characterize >95% of patients with overt scleroderma (SSc). The term ‘SSc pattern’ includes, all together, these sequential capillaroscopic changes typical to the microvascular involvement in SSc. The capillaroscopic aspects observed in dermatomyositis and in the undifferentiated connective tissue disease are generally reported as ‘SSc-like pattern’. Effectively, and early in the disease, the peripheral microangiopathy may be well recognized and studied by nailfold capillaroscopy, or better with nailfold video capillaroscopy (NVC). The early differential diagnosis between primary and secondary RP is the best advantage NVC may offer. In addition, interesting capillaroscopic changes have been observed in systemic lupus erythematosus, anti-phospholipid syndrome and Sjogren's syndrome. Further epidemiological and clinical studies are needed to better standardize the NCV patterns. In future, the evaluation of nailfold capillaroscopy in autoimmune rheumatic diseases might represent a tool for the prediction of microvascular heart involvement by considering the systemic microvascular derangement at the capillary nailfold.
Previous Section
Next Section
Introduction
The story of capillaroscopy ‘started’ from the observations of an Italian physician, Giovanni Rasori (1766–1837), who described the close relationship between conjunctival inflammation and the presence of an ‘inextricable knot of capillary loops’ by a magnifying glass [1].
During the beginning of the 20th century, Brown and O'Leary [2] used the capillaroscopic analysis to show in detail the abnormalities that characterize the involvement of microvasculature during Raynaud's phenomenon (RP) in systemic sclerosis (Ssc). In 1973, Maricq et al. [3] published the first article in Arthritis and Rheumatism, describing the specific capillaroscopic patterns in Ssc as well as the modification of the capillary blood flow during cold exposure, both in primary and secondary RP [3]. Nailfold videocapillaroscopy (NVC) represents the best method to analyse micro/cardiovascular abnormalities in rheumatic diseases. In normal conditions, the microvascular pattern is characterized by a regular array of microvessels with large intra/interindividual variability. However, absolute absence of capillary loss and giant capillaries is expected in normal pattern.
Recently, three defined major NVC patterns have been considered useful in assessing the appearance and progression of the sclerodermic microangiopathy (‘early’, ‘active’ and ‘late’ patterns) [4].
Previous Section
Next Section
The ‘scleroderma pattern’
The microvascular damage in SSc is mainly characterized by increasing structural alterations of the capillaries with progressive decrease in their density [5, 6]. Blood flow is also altered, with an average slowing of flow and increased periods of stasis.
Early in the disease, the peripheral microangiopathy may be well recognized and studied by nailfold capillaroscopy or better with the videocapillaroscopy, a non-invasive and safe technique, which is well reported to have both diagnostic and prognostic values in the presence of RP [6–11].
Previous studies have partially graded the morphological aspects of the vascular damage in patients with SSc, as assessed by nailfold capillaroscopy, and two major patterns within the term ‘scleroderma pattern’ (SSc pattern) have been recognized from the beginning: namely the ‘active’ and the ‘slow’ patterns [7].
Further morphological studies have been published more recently [12, 13].
In a recent study, microvascular alterations as detected by NVC in patients with SSc have been re-classified in three different patterns [4].
The patterns identified within the ‘SSc pattern’ include: (i) ‘Early’ NVC pattern: few enlarged/giant capillaries, few capillary haemorrhages, relatively well-preserved capillary distribution, no evident loss of capillaries (Fig. 1); (ii) ‘Active’ NVC pattern: frequent giant capillaries, frequent capillary haemorrhages, moderate loss of capillaries, mild disorganization of the capillary architecture, absent or mild ramified capillaries (Fig. 2A); and (iii) ‘Late’ NVC pattern: irregular enlargement of the capillaries, few or absent giant capillaries and haemorrhages, severe loss of capillaries with extensive avascular areas, disorganization of the normal capillary array, ramified/bushy capillaries (Fig. 1).
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FIG. 1.
A, early SSc pattern; B, active SSc pattern; C, late SSc pattern and D, normal pattern (magnification 200×).
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FIG. 2.
A, active SSc pattern (magnification 200×); B, pattern observable in patients with UCTD (magnification 200×); C, with DM (magnification 200×) and D, with APS (magnification 200×).
This study confirmed previous observations, indicating enlarged and giant capillaries together with haemorrhages as the earliest NVC finding in SSc. In the late stage of the disease, these abnormalities become rare.
Early stage is also characterized by microvessels with normal diameter coexisting with few enlarged capillaries that must be carefully investigated on all the fingers by considering the limited number of these nailfold changes during early phases of the disease [14–16].
Conversely, these changes are strongly increased in SSc patients with an ‘active’ pattern. Loss of capillaries, together with vascular architectural disorganization and ramified capillaries, were found to be rare in the early stages of SSc, whereas they seem to increase with the progression of the fibrotic phase of the disease (‘active’ and ‘late’ patterns).
A significant and gradual increase of these latter vascular abnormalities is observed during the SSc progression, and the three NVC patterns have been found to correlate with both RP and SSc durations, reflecting at least the possible evolution of the disease process [4].
While in healthy control subjects and in patients with primary RP the morphological features of the nailfold microvascular bed may remain unchanged for a long time, patients with RP associated with SSc-spectrum disorders may, on the contrary, show a higher degree of morphological variability, even after a few days.
The NVC patterns have been correlated with different clinical aspects and manifestations of SSc, as well as to the effects of treatment contributing to the overall study of the disease [17–21].
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Capillaroscopic patterns and rheumatic diseases
The presence of megacapillaries and a decreased capillary density are the hallmarks of the SSc capillary pattern, which can be detected by nailfold capillaromicroscopy.
However, in a recent large study, 186 patients with RP, 65 with undifferentiated connective tissue disease (UCTD), 47 with systemic lupus erythematosus (SLE), 26 with dermato/polymyositis, 14 with rheumatoid arthritis, 7 with primary Sjogren's syndrome (SS) and 102 patients with SSc were investigated [22].
Of the 16 patients with diffuse cutaneous SSc and the 86 with limited cutaneous SSc, 14 (87.5%) and 53 (61.6%), respectively, showed the SSc capillary pattern.
Nine of the 65 (13.8%) cases with UCTD and 24 of the 186 (12.9%) cases with RP also exhibited the same pattern.
Four of the 47 (8.5%) patients with SLE and seven of the 26 (26.9%) with dermato/polymyositis, and no patients with rheumatoid arthritis or SS exhibited the SSc capillary pattern.
The conclusion is that the ‘SSc pattern’ is often present in SSc and dermato/polymyositis. Furthermore, patients with RP and UCTD may also exhibit this pattern occasionally (Fig. 2B). Therefore, capillaromicroscopy seems to be a useful tool for the early selection of those patients who are potential candidates for developing SSc spectrum disorders.
Dermatomyositis
A defined pattern has been reported in patients affected by dermatomyositis (DM) [23]. This pattern, often associated with aspects of the SSc pattern, includes the presence of two or more of the following findings in at least two nail folds: enlargement of capillary loops, loss of capillaries, disorganization of the normal distribution of capillaries, ‘budding’ (‘bushy’) capillaries, twisted enlarged capillaries and capillary haemorrhages (extravasates) (Fig. 2C) [24].
Systemic lupus erythematosus
Characteristic SLE pattern includes morphological alterations of capillary loops, venular visibility and sludging of blood with variability of capillary loop length [25]. A recent study evaluated the association between nailfold capillary abnormalities frequently observed in SLE patients and the presence of RP, anti-U1-ribonucleoproteins (RNP) and anti-cardiolipin (aCL) antibodies [26].
One hundred SLE patients were studied. The widefield nailfold capillaroscopy was considered abnormal according to five criteria. Intercapillary distance, capillary width and capillary length were registered by videomorphometry in two fingers in all the 100 patients and in four fingers in 40 of them. Both the presence of altered capillaroscopy and that of the SSc pattern, characterized by the presence of avascular areas and enlarged or giant loops, were associated with the isolated presence of RP (P < 0.001) or anti-U1-RNP antibodies (P < 0.01), as well as with the simultaneous presence of RP and anti-U1-RNP antibodies (P < 0.001). There was a negative association between the presence of aCL antibodies and SSc pattern (P < 0.05). Higher figures for the videomorphometric parameters capillary width, intercapillary distance and capillary length were observed in patients with RP. Patients presenting both RP and anti-U1-RNP antibodies showed higher figures for intercapillary distance and capillary width. This study demonstrated significant association between nailfold capillaroscopic abnormalities and either RP or anti-U1-RNP antibodies in SLE patients. The association of RP, anti-U1-RNP antibodies and ‘SSc like’ findings on nailfold capillaroscopy (SSc pattern) in patients with SLE may suggest a new SLE subset with subclinical features of SSc.
A previous study evaluated the relationship between aCL (found in about 40–50% of patients suffering from SLE) and skin microcirculatory changes or vascular symptoms in 51 consecutive SLE patients [27].
Twenty-two patients (43.1%) had positive aCL [immunoglobins G (IgG) 22 (5–60) G phospholipids (GPL); immunoglobins M (IgM) 5 (3–16.5) M phospholipids (MPL); median titre and range, respectively] and 12 (54.5%) of them had abnormal capillaroscopic findings. In contrast, among the 29 patients without aCL, only six (20.7%) had an abnormal capillaroscopy (P = 0.027). There was no correlation between either aCL or capillaroscopy and RP. These results showed a relationship between aCL and nailfold capillary changes in patients with SLE, suggesting a direct damage of the vascular endothelium by aCL.
Anti-phospholipid syndrome
As a matter of fact, interesting microvascular alterations have been observed in patients affected by anti-phospholipid syndrome (APS). One study reported symmetrical microhaemorrhages at nailfold analysis, which were found particularly significant in patients showing the presence of both serum IgG and IgM ACL (Fig. 2D) [28]. Marked microcirculatory damage was found in relation with the occurrence of thrombotic manifestations in the APS patients in other studies, confirming the pattern [27].
A more recent study confirmed that nailfold capillary morphology is altered in patients with APS, but these changes could not be correlated to the impairment of functional parameters [29].
Sjogren's syndrome
Capillaroscopic changes have been observed also in primary SS [30]. Forty patients with SS (14 without RP, 16 with RP, 10 with ACA), 20 patients with SSc (10 with limited and 10 with diffuse disease) (disease control group) and 40 healthy controls (control group) were evaluated by nailfold capillaroscopy. Capillaroscopic abnormalities in SS ranged from non-specific findings (crossed capillaries) to more specific findings (confluent haemorrhages and pericapillary haemorrhages) or SSc-type findings. SS patients with RP presented capillary abnormalities in higher frequency than patients without RP. The majority of SS patients with ACA (80%) presented SSc-type findings. Nailfold capillaroscopy can be used as a simple non-invasive method to evaluate the microvascular abnormalities in SS patients, especially in those with RP and with ACA [31].
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Conclusions
Since in a recent study, duplex determination of the carotid elasticity or stiffness was found significantly impaired in secondary RP associated with SSc when compared with primary RP, the early capillaroscopic differential diagnosis between the two forms might improve the cardiac prognosis [32].
In fact, the recognition of the capillaroscopic patterns characterizing the secondary RP might be prognostic even for the early detection of the risk of visceral RP (i.e. coronary heart disease) [33].
In conclusion, the regular evaluation of nailfold capillaroscopy in autoimmune rheumatic diseases will represent soon a tool for the analysis even of the microvascular heart involvement by considering the systemic involvement of the microvessels in these diseases [34, 35].
