It is a multisystem disease with relapsing and remitting course. It primarily affects females of reproductive age group. It is more severe when it occurs in males and children. It has unpredictable course ranging from mild skin involvement to rapidly progressive life threatening disease. This variability in presentation poses a major challenge to diagnosis. The symptoms dramatically differ from patient to patient. The disease is characterized by spontaneous remissions and relapses. Commonly presenting features are tiredness, weight loss, excess hair loss, mouth ulcers, and joint pains. These patients cannot tolerate sunlight. They develop reddish rash over cheeks, nose, and other exposed areas on sun exposure. This is called as photosensitivity and the rash as malar rash. These manifestations appear over time as the disease progresses. It commonly affects kidney in the initial few years of diagnosis and nearly 20% may progress to end stage renal disease. In addition to kidney injury, involvement of other organs like brain, heart, lungs, muscle cause significant influence on death rates. Pregnancy in these patients creates management challenges. They may have recurrent pregnancy losses, low birth weight babies, hypertension, and fits during pregnancy. Pregnancy outcomes are improved with availability of effective treatment modalities under care of rheumatologist and obstetrician.
Diagnosis of this condition requires combination of clinical features and laboratory support. The commonly used tests are ANA, anti dsDNA, anti Sm. A positive ANA alone has poor diagnostic value in the absence of clinical features. Treatment involves a variety of approaches based on the type of organ affected.
Sun protection measures like protective clothing, use of sunscreens improve outcome by decreasing flares. Depending on the organ involved, rheumatologist choose immunosuppressive medication like steroids, azathiprine, mycophenolate, cyclosporine, tacrolimus or biologics.