SLE is a multi-organ, multi-systemic autoimmune disease predominant among females. There is high prevalence among black Americans, but its prevalence is low among black Africans.
As it is a multi systemic disease condition, its manifestation can be seen in many organ systems of the body. The symptoms can be categorized as follows.
Mucocutaneous lesions
The characteristic skin rashes are the commonest manifestation of the disease. They will have the typical malar rash over the butterfly distribution of the face. Apart from that they will presents with,
- discoid rash
- urticarial and purpuric rashes
- levido reticularis
- Alopecia (loss of hair) - they may have reversible loss of hair or scaring alopecia in which the hair does not regrow.
- They may have nasal, oral, pharyngeal and laryngeal ulcers
- Raynaud phenomenon and shincter haemorrages are seen on the hands.
The joints are usually affected and they present with migratory assymmetrical arthralgia. They may have reducible non erosiv ejoint difformity, avascular necrosis and myalgia.
Neuropsychiatric Disease
They may develop headache, fits, psychosis, strokes, aseptic meningitis, peripheral or cranial neuropathies or movement disorders.
Renal Disease
The disease can affect the interstitiam, glomeruli or the tubules. It can vary from mild (mild hematuria and proteinuria) to severe disease (nephrotic syndrome).
Cardiovascular Disease
Pericardium, myocardium or the endocardium can get affected giving rise to,
- pericarditis, cardiac tamponade
- myocardial ischemia (rare)
- endocarditis
- congenital heart block in neonatal lupus
- hypertension
Hematological Disorders
Alterations of most of the blood consituents occurs giving rise to,
- neutripenia
- lymphopenia
- thrombocytopenia
- hemolytic anemia
- thrombotic tendencies
- bleeding tendencies
- non tender lymphadenopathy
- splenomegally
Pulmonary Disease
The pleura, interstitium or the vasculature can get affected, giving rise to,
- pleuricy
- acute pneumonitis
- chronic pneumonitis
- shrinking lung
Gastrointestinal Disorders
Involvement of the gastrointestinal tract is uncommon, but they may get affected leading to,
- dyspepsia
- peptic ulcer disease
- pancreatitis
- chronic active hepatitis
- peritonitis
- mesenteric vasculitis
- The WBC/DC could be normal or low.
- High ESR and low C reactive protein ca be used to differentiate SLE from other disorders, but this is also hard as SLE patients are immunosupprsed and more prone to infections, which causes elevated CRP levels.
- High immunoglobulin levels and low complement C4 levels are also seen.
- There could be also Antinucleic antibodies and anti double strant DNA antibodies
- Mild SLE is usually traeted with tropical steroids and hydroxychloroquine. They should be also advised to avoid exposure to sunlight.
- Moderate SLE is traeted with systemic steroids - prednisilone or methyl prednisilone or steroidsparing other cytotoxic drugs.
- Cyclophosphamide pulse therapy, azathioprine, methyl prednisilone are given for other organ involvemint.
References:
Kumar and Clark’s Clinical Medicine 7th Edition
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