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  • SLICC classification criteria for systemic lupus erythematosus

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    Old 08-25-2015, 10:46 PM   #1
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    SLICC classification criteria for systemic lupus erythematosus

    Admin, please approve if OK

    Hi everyone, found this in an October 2013 Family Physician newsletter for Australia, thought that it may be of interest. Not sure if the new classification was introduced, but certainly was in the works. – thanks

    The Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) group has recently proposed a revised classification criteria, which hopes to replace the 1997 American College of Rheumatology criteria, to improve on the sensitivity and specificity. These criteria are useful and give consistency in the classification of the disease, mainly for the purpose of research and surveillance. For clinicians, these criteria also serve as a good guide and reminder of the spectrum of disease, but, in clinical practice, many patients can present in a more forme fruste or atypical state.


    Acute cutaneous lupus or subacute cutaneous lupus
    Chronic cutaneous lupus
    Oral ulcers or nasal ulcers
    Non-scarring alopecia
    Synovitis involving two or more joints
    Haemolytic anaemia
    Leukopenia (<4 000/mm3) or lymphopenia (1 000/mm3)
    Thrombocytopenia (<100 000/mm3)


    Low complement
    Direct Coombs’ test
    Antiphospholipid antibodies

    Diagnosis of definitive SLE requires four or more criteria, with at least one clinical and one laboratory, with the exception of biopsy-proven LN (which requires fewer criteria). Criteria are cumulative and need not be present concurrently



    Constitutional manifestations such as malaise, fatigue, fever and weight loss affect most patients at some time during their disease. They are, however, non-specific, and other non-SLE or non-rheumatological conditions (eg. infection and malignancy) can present the same way. Fibromyalgia is commonly observed in patients with SLE, but is considered a non-inflammatory complication of the disease. More sinister constitutional symptoms such as fever and weight loss warrant investigation before being attributed to lupus.


    Up to 95% of patients with SLE have intermittent arthritis. The most common presentation is a symmetrical polyarthritis, affecting hands, wrists and knees. The degree of swelling is less prominent than seen in rheumatoid arthritis. Tenosynovitis is a relatively common musculoskeletal manifestation. Joint deformities are uncommon, as are erosive changes on X-rays. Myalgia is also a common manifestation, even though true myositis is relatively rare.

    Cutaneous manifestations

    The spectrum of cutaneous manifestations of lupus erythematosus (LE) is broad, but the most classical forms associated with systemic LE are the acute malar and chronic discoid lupus erythematosus (DLE) rash. Both can be quite photosensitive in nature.

    Acute malar rash is a slightly raised erythematous rash of the face, particularly cheeks and nose, with nasolabial sparing, known as the ‘butterfly’ rash. A worsening of the rash usually accompanies a flare of systemic disease. Sometimes a more generalised form over the body is present.

    DLE are characterised by slightly raised, scaly lesions that have a potential to scar. They can be found commonly on the scalp and face, and less commonly over the limbs and trunk. Only 5% of people with DLE have SLE, but conversely among individuals with SLE, 20% will have DLE.

    Subacute cutaneous lupus erythematosus is another lupus-specific rash. It is a common skin manifestation in drug-induced lupus. The rash is extremely photosensitive. The two main variants look as their names suggest: papulosquamous looks psoriasiform, and annular polycyclic gives a characteristic ring pattern.

    Other cutaneous manifestations such as alopecia, oral ulcers, Raynaud’s phenomenon, urticaria, lichen planus, vasculitis or nail fold infarcts are LE non-specific, but they often present at times when patients experience increased lupus disease activity.

    Renal manifestations

    Lupus nephritis (LN) is one of the more serious manifestations, and contributes significantly to mortality. It occurs in 30–50% of SLE patients during their disease course. It is important to recognise that LN can be relatively ‘silent’, and symptoms are often driven by other organ involvement or non-specific constitutional symptoms. Renal involvement can be missed if urinalysis is not performed. Definitive diagnosis and information on prognosis can be obtained by renal biopsy, but presence of glomerular haematuria, proteinuria or casts, are the key features of LN.

    Haematologic manifestations

    The most frequent haematological manifestation of SLE is anaemia. This is usually due to chronic inflammation, but sometimes an autoimmune haemolytic anaemia can be demonstrated. Leukopenia, such as lymphopenia or less commonly neutropenia, is also well recognised, but it rarely predisposes to infections. Thrombocytopenia may also be a recurring feature.

    Serosal manifestations

    Pleural and pericardial inflammation can occur during active SLE. They can present as pleuritic chest pain, when associated with pleurisy or pericarditis. Less commonly, the presence of pleural or pericardial effusion may present with pleuritic chest pain with or without exertional dyspnoea. This symptom adds robustness to the criteria for identifying SLE. Other cardiopulmonary manifestations such as interstitial lung disease, pulmonary hypertension or Libman–Sacks endocarditis are less common complications of the disease.


    Antinuclear antibodies

    The antinuclear antibodies (ANA) test is the serological hallmark of SLE. Up to 98% of patients with SLE will have a positive ANA, making it highly sensitive and useful as a screening test. A negative ANA makes SLE very unlikely and other diagnoses should be sought to explain symptoms.

    Both titre and pattern are relevant. A titre of 320 or greater is considered clinically significant. Low-titre ANA can be found in an otherwise healthy population. The most common patterns observed in SLE are homogenous and speckled pattern while other patterns may be associated with other connective tissue disease. While the titre of ANA can fluctuate over time, it is not useful to repeat ANA testing unless there is still a question regarding the diagnosis. The finding of a positive ANA must be taken into context of the clinical manifestations. A positive ANA in the absence of the clinical features associated with connective tissue disease may be irrelevant. The test has generally poor specificity, and many autoimmune or non-autoimmune conditions can also be associated with a positive ANA.

    Last edited by zoe305; 08-27-2015 at 03:15 PM.

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    Old 08-27-2015, 08:15 AM   #2
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    Re: SLICC classification criteria for systemic lupus erythematosus

    This is a very good summary of SLE findings. It would be helpful to have this posted as a sticky post at the top of the lupus board, if administrators approve. This is much more comprehensive than the ACR criteria, and helpful for people seeking diagnosis to see the wide extent of symptoms possible. Thanks for posting this!

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