Sammendrag
Most cases of diffuse alveolar hemorrhage syndromes are associated with autoimmune diseases. Early recognition is crucial, because prompt institution of treatment is required for survival. We present a case with an overlap syndrome with features of both systemic lupus erythematosus and Sjogren�s syndrome. A 13 year-old girl was admitted to hospital because of increasing exercise dyspnea, coughing and paleness. One year earlier she had an acute parotitis on the right side, and she had continual bilateral parotid swellings ever since. She was otherwise previously healthy. There was no family history of autoimmune diseases. On physical examination she was pale with bilateral parotid swellings. She had iron deficiency anemia (Hgb 5,8 g/dl), weakly positive Coombs� test, hyperglobulinemia, slightly increased amylase, strongly positive ANA, anti-SSA and anti-SSB and weakly positive anti-RNP. MRI of the parotid glands showed swellings and multiple microcysts bilaterally. Histologic examination of the parotids revealed ductectasia and chronic inflammation. CT of the chest showed bilateral interstitial infiltrates with ground-glass opacities. Bronchoscopic examination showed hemorrhagic and ecchymotic lesions with ongoing bleeding. She was treatedwith methylprednisolon pulses, iv-cyclophosphamide, oral corticosteroids, methotrexate, hydroxychlorochin and iron supplementation. Except for one episode of rebleeding with severe dyspnoea and hemoptysis, she has remained stable on weekly pulses the last two months. This case illustrates a rare, but potentially life-threatening complication of Sjogren/SLE overlap syndrome.
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