An aggressive and lethal course of Churg-Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy.
Churg-Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.
Çiledağ
Aydin
A
Department of Chest Diseases, Ankara University School of Medicine, 06100 Cebeci, Ankara, Turkey.
Deniz
Hülya
H
Eledağ
Serpil
S
Özkal
Canan
C
Düzgün
Nurşen
N
Erekul
Selim
S
Karnak
Demet
D
D000328:Adult; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D017809:Fatal Outcome; D006333:Heart Failure / Q000209:etiology* / Q000473:pathology; D006470:Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D007416:Intestinal Perforation / Q000209:etiology* / Q000473:pathology; D008297:Male; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D011650:Pulmonary Alveoli / Q000098:blood supply; D011859:Radiography