Necrotizing sarcoidosis presenting with hemoptysis

Clinical History

43-year-old non-smoker presenting with acute hemoptysis in the emergency department.

Imaging Findings

A 43-year-old social worker presented with minor hemoptysis of two months duration. He denied other respiratory symptoms, fever, night sweats or weight loss. Four years ago he had been diagnosed having sarcoidosis with bihilar lymphadenopathy and parenchymal opacification. A Chest X-ray from 1997 showed reticulo-nodular pattern with predominantly perihilar distribution and only faint bihilar lymphadenopathy (Figure 1a). Because of a troublesome cough at that time he had been treated successfully with systemic corticosteroids for a year. The physical examination at the current presentation was unremarkable. Pulmonary function tests and the laboratory findings (hematogram, liver enzymes, calcium) were normal. The actual chest X-ray showed severe bilateral lymphadenopathy and parenchymal opacification, most pronounced in the right upper lobe, where cavitation was suspected. The CT scan of the thorax confirmed cavitation as well as bilateral hilar and mediastinal lymphadenopathy with calcification. HR-CT showed micronoduli with peribronchovasvular distribution. Bronchoscopy revealed no abnormalities. Neither microorganisms nor malignant cells were found in the bronchoalveolar lavage. A systemic corticosteroid therapy was started. Promptly hemoptysis stopped and bilateral opacification decreased.

Discussion

Hemoptysis allows a wide range of differential diagnosis: Infection, including tuberculosis, chronic bronchitis, bronchiectasis and aspergilloma. Vascular disorders including arterio-venous malformations, venous hypertension, pulmonary embolism, bronchial wall injury including foreign body erosion and bronchoscopy / biopsy, tumors including carcinoma and benign tumors. Additionally, rare causes of hemoptysis should be taken into account such as Wegener’s granulomatosis and sarcoidosis. The presence of severe bihilar lymphadenopathy with calcifications, bilateral pulmonary consolidations and the presence of interstitial nodules with peribronchovascular distribution together with the absence of microorganisms and malignant cells in the broncho-alveolar lavage suggested necrotizing sarcoidosis as the cause of hemoptysis in the present case. Bleeding rarely occurs in sarcoidosis and can present as diffuse alveolar hemorrhage (1, 3) with or without hemoptysis (2-4). Alveolar hemorrhage may occur even in absence of severe pulmonary involvement by sarcoidosis (1) whereas hemoptysis is mostly seen in cases with massive pulmonary fibrosis and cavitation (2-4 ). In these cases bronchopulmonary aspergillosis or aspergilloma may be associated with sarcoidosis and cause hemorrhage (4). No endobronchial aspergillus infection was detected during bronchoscopy / BAL in the present case. Hemoptysis in sarcoidosis is often a sign of end stage disease and may end fatal. In severe cases of hemorrhage bronchial artery embolization or even surgery may be necessary for treatment(5). However, the clinical course in the present case was benign with rapid clinical recovery and improvement of the imaging findings after starting corticosteroid therapy.

Differential Diagnosis List

Final Diagnosis

Necrotizing sarcoidosis presenting with hemoptysis

URL:	https://www.eurorad.org/case/793
DOI:	10.1594/EURORAD/CASE.793
ISSN:	1563-4086