Systemic lupus erythematosus with interstitial pneumonitis and systemic angiitis

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A case of SLE with simultaneous occurrance of interstitial pneumonitis and systemic angiitis after administration of antibiotics and antituberculous drugs is reported.A 24-year-old woman developed fever and arthralgia during her first pregnancy. On admission in April 1980, she had a mild proteinuria, casturia, hypergammaglobulinemia, positive antinuclear antibodies, high titers of anti-DNA antibody and hypocomplementemia. Circulating immune complex was positive. Renal biopsy revealed mild mesangial proliferation and wire loop lesions in glomeruli. A diagnosis of SLE was made and prednisolone 40 mg daily was effective. In March 1982, fever relapsed when prednisolone was tapered to 10 mg daily. On the second admission, facial erythema was noted. Anti-DNA antibody was 304 U/ml (normal 10 U/ml), CH50 13.7 U/ml, CRP 2+ and the erythrocyte sedimentation rate 54 mm per hour. Prednisolone was increased to 30 ml daily. Fever subsided and titers of anti-DNA antibody decreased. In May 1982, fever, mononeuritis multiplex, dry cough and mild dyspnea developed when she was treated with Cefaclor for a carious tooth. In June, combination therapy with SM, INH and RFP was added because another SLE patient in the same ward was discovered to have pulmonary tuberculosis. Soon after psychosis, tremor and rigidity developed and dyspnea worsened. Blood gas analysis revealed severe hypoxemia and an X-ray film of the chest revealed diffuse interstitial infiltration in both lungs. Interstitial pneumonitis, CNS lupus and systemic angiitis were suspected. Pulse therapy with methylprednisolone was started with rapid improvement of hypoxemia and neurological symptoms. Interstitial pneumonitis relapsed again after slight reduction of prednisolone. The patient died after massive gastrointerstinal bleeding and anuria.At autopsy, subacute interstitial pneumonitis was observed in both lungs without evidence of cytomegalic inclusion body, Pneumocystis carinii or other opportunistic infections. Generalized angiitis in healing phase with adventitial fibrosis, intimal thickening, mural thrombosis, and ruptured internal elastic laminae was found in small arteries of kidneys, heart, liver, pancreas, spleen, small intestine, adrenal glands and ovaries. However, no evidence of angiitis was found in pulmonary arteries. Ruptured internal elastic laminae were found in the main coronary arteries.This case represents a rare combination of interstitial pneumonitis and systemic angiitis in SLE, in which antibiotics and antituberculous drugs were implicated as triggering factors.
日本臨床免疫学会の論文

Systemic lupus erythematosus with interstitial pneumonitis and systemic angiitis

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