Sinonasal Sarcoidosis: A report of four biopsy-proven cases

Introduction and aim: Sarcoidosis is a chronic systemic disease of unknown etiology characterized by non-caseating inflammation involving one or more organs with predilection for pulmonary and upper respiratory tract involvement. It presents with a variety of signs and symptoms which can be generalized or focused on a single organ. There are only limited data available regarding the frequency of sinonasal involvement. The symptoms of patients with sinonasal sarcoidosis resemble the symptoms of chronic rhinosinusitis. The aim of this paper is to discuss the diagnosis, management of sinonasal sarcoidosis and to present four cases. Materials and methods: We retrospectively analyzed 4 cases of biopsy-proven sinonasal sarcoidosis. Two patients presenting with symptoms of sinonasal disease were identified by the treating pulmonologist. Two patients presented first at the department of ENT surgery with sinonasal symptoms. Results: The charts of 4 patients with biopsy-proven sinonasal sarcoidosis were reviewed. Follow-up time ranged from 7 to 88 months in our ENT departement. The study population included 2 males and 2 females. The most frequent signs and symptoms were nasal obstruction, rhinorrhea and headache. Pulmonary sarcoidosis was radiologically staged, 2 of our patients presented pulmonary sarcoidosis, 1 patient stage I and 1 patient stage II. In addition to sinonasal and pulmonary involvement sarcoidosis affected other organs in 2 patients. The levels of the angiotensin-converting enzyme (ACE) in serum where elevated in 2 patients. Spirometry was normal in 3 of our patients. CT scan of the paranasal sinuses showed in all patients signs of chronic sinus inflammation with mucosal thickening and opacification of one or more sinuses. The histopathological reports of the tissue biopsies revealed typical granulomatous inflammation in all cases. In 3 of our patients endoscopic sinus surgery was performed with a good outcome. Conclusion: Sinonasal sarcoidosis is a rare disease manifestation of sarcoidosis. It can be isolated or associated with a multisystemic, particulary pulmonary granulomatous disease. The prevalence of sinonasal sarcoidosis remains difficult to estimate. Biopsy with histopathological examination needs to be performed to confirm the diagnosis. In literature there is no consensus on treatment of patients with sinonasal sarcoidosis.