Diagnostic criteria for sarcoidosis of the sinuses.

Background: Although newer technologies facilitate its diagnosis and treatment, little is known about sarcoidosis of the paranasal sinuses.

Objectives: We sought to better characterize sarcoidosis of the sinuses and establish criteria for diagnosis.

Methods: Case-finding criteria were established, and over 50 reports of sarcoidosis of the sinuses in the medical literature were reviewed. Nine case reports of patients fulfilling the case-finding criteria were identified, as were 6 additional patients from our clinics.

Results: Nasal obstruction and chronic sinusitis were the usual initial complaints from patients and were associated with mucosal crusting, studding, plaque-like changes, or polyps in the nose in 5 of 6 of our patients. The most consistent finding in nose and sinuses was an erythematous, edematous, friable, hypertrophied mucosa. Like 5 of 6 of our patients, most patients had extra-pulmonary sarcoidosis involving multiple organs, but some had isolated upper respiratory disease. Radiologic studies showed extensive and often complete opacification of the sinuses and nose similar to that seen in diffuse polyposis associated with chronic bacterial and fungal sinusitis. No specific histopathologic findings distinguished sinus disease from those reported with pulmonary involvement. Pharyngeal involvement was present in 2 case reports and caused the apparent asphyxiation of 1 of our patients.

Conclusion: Sarcoidosis of the sinuses should be considered in the differential diagnosis of sinusitis, especially in association with nasal polyposis, even when the sarcoidosis has not been otherwise diagnosed. On the basis of this experience, we propose diagnostic criteria for sarcoidosis of the sinuses. These include (1) radiologic evidence of sinusitis, (2) histopathologic confirmation of noncaseating granuloma in the sinus tissue supported by negative stains for fungus and acid-fast bacilli, (3) negative serologic test results for syphilis and antineutrophil cytoplasmic antibodies, and (4) no clinical evidence of other disease processes associated with granulomatous nasal and sinus inflammation. These criteria will provide the basis for further studies to assess both the natural history and the effectiveness of treatment in sarcoidosis of the sinuses.