RESUMO
Aims: Airway involvement is a common feature of sarcoidosis and mucosal abnormalities may be evident in the respiratory tract. However, firm data establishing the clinical features and prognosis of sarcoidosis in these patients is lacking although the incidence of endobronchial disease is high. The purpose of this study was to evaluate the clinical features of the patients with limited, diffuse and no endobronchial involvement. Another aim was to investigate the prognostic differences between these patients. Methods: We conducted a retrospective study to evaluate the clinical and laboratory findings of 48 patients with endobronchial sarcoidosis and 50 patients without endobronchial involvement seen at our institution. The patients fulfilled the clinical, radiologic or both features of sarcoidosis supported by the histopathologic evidence of noncaseiting granulomas. Six to ten bronchial biopsies were taken from each patient. The sample was considered positive if it demonstrated noncaseiting granulomas with negative fungal and mycobacterial cultures. The patients were classified into three groups according to the histopathologic biopsy results: 1) No endobronchial involvement, 2) Limited endobronchial involvement: One biopsy site positive and 3) Diffuse endobronchial involvement: Two or more biopsy sites positive for noncaseaiting granulomas. Results: Bronchial biopsy was positive in 82% of the abnormal appearing airways while it was diagnostic in 36% of the normal appearing mucosa. The most frequent bronchoscopic appearence was miliary infiltration. Nodular, erythematous lesions and edematous mucosal swelling were other bronchoscopic findings. There were no significant differences between the three groups for FEV1, FVC, TLC, DLCO/VA serum and 24 h urinary calcium levels. Serum ACE levels were significantly higher (p<0.001) in patients with limited and diffuse bronchial involvement compared to patients with no endobronchial disease. The extrapulmonary organ involvement (p<0.001) and progressive disease incidence was more frequent (p<0.001) in patients with limited and diffuse endobronchial disease. Conclusions: Endobronchial involvement in sarcoidosis appears to be a significant predictive risk factor for progressive disease. Patients with limited or diffuse endobronchial disease have more severe extrapulmonary organ involvement and a worse prognosis than patients without endobronchial disease. Bronchoscopy may identify such patients carrying a risk factor for progressive sarcoidosis.
RESUMO
Sarcoidosis is a systemic granulomatous disease of unknown etiology that can involve nearly all organs. Liver is the third most commonly affected organ following the lungs and the lymph nodes. Cases with liver sarcoidosis are usually silent clinically while a few can progress to cirrhosis and portal hypertension in less than 1% of the patients. A 56 year old female was referred for ecchymosis, protuberant abdomen, bilateral pretibial and ankle edema. Medical history did not reveal any previous disease. Ascites, hepatomegaly, splenomegaly, and facial telengiectasies were present on physical examination. Chest x-ray and CT were normal. Papules and plaques on the knees developed six days after admission. Skin biopsy revealed granulomatous dermatitis. Serum ACE was 250 IU/L. Liver biopsy showed non-caseiting granulomas, severe hepatitis and fibrosis. Sarcoidosis was confirmed based on high serum ACE, histopathologic findings of the skin and liver biopsy samples that revealed non-caseiting granulomas. We report a case of sarcoidosis complicated by cirrhosis as the initial manifestation of the disease without lung involvement. An extensive literature review of sarcoidosis, focusing on case reports, which presented with cirrhosis and portal hypertension without lung involvement, was made.