Following is the summery of symptoms by a doctor :-
• Uncontrolled hypertension with some form of membrano-proliferative glomerulonephritis with predominant IgA and complement staining ? IgA nephropathy (UNUSUAL – absence of significant microscopic haematuria)
• Small-vessel leukocytoclastic vasculitis – clinically rather typical Henoch-Schönlein’s purpura (of adult age). (No clinical features of microscopic polyangiitis).
• CLINICAL SUMMARY: Background of anxiety and tension; moderately severe hypertension on treatment since 1998 (father hypertensive) - UNCONTROLLED.
• ‘Palpable purpura’ in a rather typical Henoch-Schönlein’s distribution since 1999-00; but no joint, or abdominal symptoms.
• Increasing proteinuria (highest ~ 2-3 gm/24 hr; latest [28/12/02]: 1.6 gm; but no significant RBCs in urine with renal biopsy (6/11/00) Predominantly peripheral IgA and granular complement staining reported – interpreted as ‘membrano-proliferative GN’ – my feeling is that it fits with IgA nephropathy often with HSP type skin lesions (Unusual feature is absence of significant haematuria).
• No features of any systemic vasculitis. Never had high WBC (except one reading of ~11,000/cmm) or high ESR or high platelets, no features of systemic inflammation (A/G ratio etc).
• No clinical features of microscopic polyangiitis. ANA, anti-DNA negative; C3, C4 normal (unfortunately IFT-ANCA not done) only ELISA (MPO and
PR-3 – both negative).
Summary of prior medications; treatments; hospitalization/procedures: Mainly anti-hypertensives: presently prazocin 2.5 (AM) and 5 mg (PM) + ramipril 10 mg daily – BP NOT well controlled.
Physical examination: Severe hypertension and HSP-type skin lesions. MSK exam at the first contact: Normal
Plan:
1. Reassurance that he is NOT suffering from any serious systemic vasculitis.
2. That his main problem is UNCONTROLLED HIGH BLOOD PRESSURE that needs to be appropriately controlled by the specialists in this problem.
Onset: Insidious.
Course:
• Noted to have borderline hypertension for the last several years, started on regular drug treatment from 1998 (after an ‘Executive Check-up’ at Apollo Hospital).
• Noted to have red pin-head spots on the lower extremities going up to the gluteal region.
• Approximately the same time found to have trace then slowly increasing proteinuria for which a renal biopsy was done at Apollo Hospital (6/11/00: Apollo Hospital - ‘membranoproliferative GN’ with predominant IgA and complement staining.
Present problem:
• Urine showing increase in 24 hr protein.
• ‘Purpuric’ rashes on the dependent regions – lower extremities; nothing very severe but, – concerned about ‘vasculitis’ – some immunological disease.
Review of systems and direct questions:
Denies: Features suggestive of systemic inflammatory rheumatic disease (e.g. significant early morning stiffness; constitutional symptoms, and / or improvement of symptoms on gentle use of joints). Denies: Lesions of psoriasis. Denies: any skin disease except ring worm and the purpuric lesions (HPI). Denies: skin-mucosal lesions suggestive of connective tissue disease (oral ulcers, scalp lesions; photosensitivity, facial rash, rash on the exposed parts of the body; hardening of the skin, rash on the extensor surfaces of the joints [Gottron’s papules]). Denies: HEENT diseases, features suggestive of sicca syndrome. EXCEPT deviated septum with frequent ‘colds’. Denies: Features suggestive of entrapment syndromes. Denies: Features suggestive of inflammatory bowel disease, acute infective diarrhoea. Denies: Features suggestive of recurrent urethritis. Denies: Limb-girdle muscular weakness, food regurgitating from the nose. Denies: Features suggestive of Raynaud’s / vasospastic phenomenon. Denies: Features suggestive of oesophageal involvement due to connective tissue diseases. Complaints of: Features suggestive of any renal disease – proteinuria – see HPI. Denies: History of diabetes mellitus. Complaints of: hypertension – see HPI. Denies: Features suggestive of any cardiovascular disease. Denies: Features suggestive of any pulmonary disease. Denies: Abdominal pain, swelling, distension. Denies: Features suggestive of any neurological problems. Denies: Allergy. Complains of: anxiety and tension; on occasions starts shaking, feels very nervous.
Past, social and personal history:
Married with 2 children. Ex-smoker (gave up 10 yr ago after 10 years of 1 pack per day). Minimal alcohol consumer, now mainly a teetotaller. No major medical or surgical illnesses.
Family history: Father diabetes mellitus, mother bipolar ailment with painful knees (OA), brother has some type of Sun sensitivity. Sister is well. Denies: joint diseases, back problem, chronic skin condition, thyroid disease, any other autoimmune disease.
Medicines and medical advice taken in the past / present: Ramipril, indapamide, amlodipine, enalapril, dipyridamole, prazocin, fexofenadine. At present presently prazocin 2.5 (AM) and 5 mg (PM) + ramipril 10 mg daily – BP NOT well controlled.
Physical Examination:
General physical examination: Appearance and attitude: A tall well build man, walked in the clinic normally. Afebrile, normal respiration. In no distress. Skin showed small-vessel vasculitic ‘palpable purpuric’ lesions on the legs, thighs and buttocks – sparse not too many. A few such lesions were also present on the posterior surface of the upper arms bilaterally. The mucosa, HEENT, neck-thyroid-JVP, LN, normal. No paedal oedema.
Vitals:
Date Blood pressure mm Hg Pulse / respiration Weight Kg / Height meters BMI
6/1/03 158/116 99/1.83 29.56
Systemic examination: Cardiovascular system: Normal heart sounds without any extra sounds, murmurs or rub. Respiratory system: Normal vesicular breathing without any adventitious sounds or rub. Abdomen: Normal with normal skin, no distension, tenderness or organomegaly. Neurological system: Higher functions normal, normal cranial nerves, motor and sensory systems with normal reflexes.
Rheumatological examination:
Gait Normal. Arms: joints normal with no tenderness, swelling, effusion; with full range of movements, normal alignment and good stability.. Legs: joints normal with no tenderness, swelling, effusion; with full range of movements, normal alignment and good stability.. Spine: normal with no tenderness with full range of movements, normal alignment and good stability.
Relevant investigation reports:
Date ESR
mm 1st hr (Wstn) Platelets
X 109/l WBC/Diff
Cmm/dl; % Hbg
Gm/dl A/G ratio AST/ALT/ALP IU Urea / Crtn mg/dl Blood glucose mg / ld Urine
Routine
Micrsp
25/1/02 5 198 7000;G74-L24% 13.1 3.5/2.5 28/32/10.58 KA 31/0.85 60 Alb. ++
28/12/02 20/1.20 Prt. Trace
1/11/00 15 263 11800;G71-L18% 16.0 /-/-131 30/- r
31/10/00 264 4.8/3.8 22/24/131 30/1.4 Prt. ++
9/4/00 Prt. +++
3/3/00 10 249 7300;G56-L35% 13.6 79
22/10/99 Prt. ++
3/7/98 3 215 6700;g59-l30% 15.1 5.1/2.7 -/33/126 84 Alb. Trace
Chole-sterol mg/dl Tri-glyceride mg/dl HDL mg/dl LDL mg/dl VLDL
Mg/dl Uric acid mg/dl T3 ng/dL T4 µgm/dL TSH µunit/ml
26/10/99 3.5 1.0 2.2
Histopathology: Nasal mucosal biopsy: (6/11/00): No significant pathology.
Immunological tests: Anti-GBM (9/11/00): negative.
Date ANA titre/IU Anti-DNA titre/IU Anti-ENA C3/C4 mg/dL RF titre/IU CRP mg/dL GPL/MPL ANCA
6/11/00 -ve -ve
31/10/00 146/79 (Both N)
13/4/00 -ve Both types NEG
Radiological findings:
Routine chest, sinuses, ultrasound abdomen etc: all normal.