Systemic lupus erythematosus (SLE or lupus)
SLE is a chronic autoimmune disease that could affect any part of the body. It is an unpredictable disease with a relapsing (periods called flares) and remitting pattern. Symptoms not only differ from person to person, but also from one flare to the next. A multitude of symptoms could result depending on the location of disease activity. Common initial and chronic complaints include malaise, fever, chronic fatigue, joint pain affecting multiple joints and muscle pain.Oher symptoms include:
- Dermatological: malar rash, alopecia (hair loss), ulcers, discoid rash (red, thick scaly patches) or other non-specific rashes
- Musculoskeletal: joint pain (arthritis and arthralgia) and muscle pain
- Neurological: Seizures, psychosis, headaches
- Renal: hematuria, proteinuria, chronic renal impairment (chronic kidney disease)
- Cardiovascular: pericarditis, myocarditis, endocarditis, vasculitis
- Respiratory / pulmonary: pleuritis, pleural effusion, pneumonitis,
- Hematological: anaemia, low platelet count, low white blood cell count, Hughes Syndrome
ACR (American College of Rheumatology) Diagnostic Criteria
The ACR criteria was established in 1982 and revised in 1997 as inclusion criteria for research studies. It was not intended to be used as diagnostic criteria, but the lack of diagnostic criteria has inevitably led to the adoption of the ACR criteria for diagnostic purposes, despite the fact that these criteria do not do well in such capacity. For inclusion in clinical trials, patients must present with at least four of the below mentioned symptoms, either simultaneously or serially during a given period of observation.
1. Malar rash (rash on cheeks)
2. Discoid lupus (red, scaly patches on skin which cause scarring)
3. Photosensitivity (exposure to ultraviolet light causes rash)
4. Oral or nasopharyngeal ulcers
5. Arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling or effusion
6. Renal disorder: More than 0.5g per day protein in urine, or cellular casts seen in urine under a microscope.
7. Neurologic disorder: Seizures or psychosis
8. Serositis: Pleuritis (inflammation of the membrane around the lungs) or pericarditis (inflammation of the membrane around the heart)
9. Hematologic disorder: Haemolytic anaemia (low red blood cell count) or leukopenia (white blood cell count<4000/ul), lymphopenia ( <1500/ul ) or thrombocytopenia (<100000/uL) in the absence of offending drug.
10. Anti-nuclear antibody test positive , very sensitive (95 - 98%) but non specific.
11. Immunologic disorder: Positive anti-Sm DNA, anti-ds DNA, anti-phospholipid antibody and/or false positive serological test for syphilis, presence of anti-ss DNA in 70% of patients (though also positive in patients with rheumatic disease and healthy persons)
St. Thomas Hospital Criteria
Dr Graham Hughes, a medical authority on lupus, has published a set of alternative criteria in 1982 (commonly known as the St. Thomas Hospital criteria) to diagnose SLE:
1. Teenage "growing pains": Growing pains in the UK, is a label widely used for joint pains in teenagers and seems to cover a spectrum of rheumatology from arthritis variants through to lupus.
2. Teenage migraine: Headache, cluster headache and migraine can be encountered and a strong history of teenage migraine may be of lupus significance, either at that time or subsequently.
3. Teenage "glandular fever": Prolonged teenage glandular fever is a label which crops up time and time again in lupus patients and prolonged periods off school in many SLE patients is a recurrent theme.
4. Severe reaction to insect bites: This is a feature of so many lupus patients. Not only are they susceptible to insect bites but often reactions are severe and prolonged - the skin is a major organ affected by lupus.
5. Recurrent miscarriages: Lupus itself seems not to be a cause of recurrent miscarriage but where the anti-phospholipid syndrome (APS) is present, recurrent spontaneous fetal loss can be significant.
6. Premenstrual exacerbations: Although difficult to quantify, it is believed that significant pre-menstrual disease flare is sufficiently prominent in lupus to be included in this list. All rheumatic diseases are clinically influenced by the menstrual cycle.
7. Septrin (and sulphonamide allergy): Adverse reactions to these drugs is quite common in lupus and the clinical onset of the disease may have coincided with the use of e.g. Septrin.
8. Agoraphobia: Agoraphobia/claustrophobia are often present at a time when lupus disease is active. A history of these conditions can be protracted, lasting for months or even years. In many cases the history is not volunteered or the episodes are in the interim considered unrelated to lupus.
9. Finger Flexor Tendonitis: Arthralgia and tenosynovitis are common features in lupus and although not specific, the finding of mild to moderate ten-finger flexor synovitis is a useful pointer in the presence of other lupus features. It is subtly yet significantly different in pattern from other arthritic diseases.
10. Family history of autoimmune disease: As the genetics and statistics of the various autoimmune diseases become better defined, the strength of a particular family history will become more precise. The family history is important, as lupus is genetically determined.
11. Dry Shirmer's Test: A "bone dry" Shirmer's Test (levels of eye moisture) points towards one of the autoimmune diseases and in the patient with vague or nonspecific symptoms is worth its weight in gold.
12. Borderline C4: Genetic complement deficiencies have been known to be associated with lupus for over three decades and in the diagnostically difficult patient, especially where a family history is present, borderline C4 levels can be significant indicators.
13. Normal CRP with raised ESR: An important diagnostic aid. A very low CRP in an otherwise inflammatory situation is strongly supportive of lupus or primary Sjögren's Syndrome.
14. Lymphopenia: In the patient with non-specific complaints and unremarkable blood tests, a borderline or low lymph count can be overlooked. It can be common in lupus and is certainly worth inclusion among minor criteria.
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