Sinonasal sarcoidosis: review and report of fifteen cases

Abstract

Objectives: Sinonasal sarcoidosis remains a poorly understood and uncommon chronic granulomatous disease of unclear origin. We have attempted to characterize the main clinical and radiologic criteria for diagnosis and to discuss the treatment.

Methods: A retrospective study of 15 cases of chronic, symptomatic, and biopsy-proven sinonasal sarcoidosis and a review of the literature are realized.

Results: Among the 15 patients, there were 8 women and 7 men with a mean age of 44 years. The most frequent presentation was a chronic, often crusty, rarely destructive inflammatory rhinosinusitis with nodules on the septum and/or the turbinates. Pulmonary sarcoidosis was associated in 12 cases. Involvement of the nasopharynx, the pharyngolarynx, the skin, the lachrymal and salivary glands, and the liver was associated in some cases. Levels of angiotensin-converting enzyme were elevated in 10 cases and normal in 3 cases. Gallium scan performed in three cases was positive. Radiologic studies showed nodules on the septum and/or the turbinates in 14 cases, complete or subtotal opacification of the sinuses and/or the nasal cavities in 13 cases, and nasopharyngeal or pharyngolaryngeal lesions in 4 cases. Treatment with corticosteroids, methotrexate, azathioprine, and surgery appear globally disappointing in view of the side effects and the relapses during a long follow-up (3-15 yr; mean, 6 yr).

Conclusion: On the basis of this study, we propose the following diagnostic criteria: 1) histopathologic confirmation of noncaseating granuloma; 2) chronic rhinosinusitis poorly responsive to conventional treatment and radiologic evidence of rhinosinusitis, often with nodules on the septum and/or the turbinates; 3) elevated level of angiotensin-converting enzyme; 4) positive gallium scan (if performed); 5) frequent evidence of systemic, especially pulmonary, sarcoidosis; 6) no evidence of other granulomatous diseases, such as Wegener granulomatosis.