Sarcoidosis is a noncaseating granulomatous disease, oftentimes involving multiple systems (Fig. 31.1
). A demographic predisposition for African American and Scandinavian individuals of age range in the twenties and thirties has been demonstrated (17
Development is likely multifactorial, leading to an abnormal immune response, of which T cells and their proinflammatory cascades are thought to be at the center. Mycobacterial tuberculosis catalase-peroxidase protein was reported to be present in approximately 50% of sarcoidosis tissue samples (18
). Activated tissue macrophages are the primary source for angiotensin-converting enzyme elevations (19
). Genetics appear to contribute to predisposition of development of sarcoidosis. In one study involving 210 pairs of monozygotic and dizygotic twins and at least one proband, concordance rates of sarcoidosis were found to be significantly higher in monozygotic than in dizygotic twins. Compared with the general population, there was an 80-fold increased risk of developing sarcoidosis in cotwins of effected monozygotic brothers or sisters (20
). Responsible genes, such as butyrophilin-like 2 gene, have been proposed (19
). It is hypothesized that an environmental factor prompts an aberrant immunologic response, though no strong associations in the literature have led to a specific environmental offender.
The wide range of symptomatology makes recognition of sarcoidosis a challenge. Incidental discovery on chest x-ray (CXR) in asymptomatic patients accounts for a large proportion of presentations. In the symptomatic patient, one or more of the following systems may be involved: respiratory (cough, dyspnea, chest pain), integumentary (lupus pernio, erythema nodosum, papules, nodules, plaques), ocular (orbital pain, uveitis, visual changes), rheumatologic (arthralgia, myalgia), and constitutional (fevers, night sweats, fatigue) (21
). Lupus pernio, the most characteristic skin lesion for sarcoidosis, occurs at a higher preponderance in sarcoidosis with sinonasal features (Fig. 31.2
As pulmonary findings are a hallmark of sarcoidosis, a close collaboration with a pulmonologist is recommended. Chest radiographic findings are classified as follows: stage 0, normal CXR; stage 1, isolated intrathoracic adenopathy; stage 2, intrathoracic adenopathy and parenchymal disease; stage 3, parenchymal disease; and stage 4, pulmonary fibrosis (22
Sinonasal manifestations occur in approximately 1% to 5% of cases, of which only 10% are present in absence of pulmonary disease (23
), and include nasal congestion, nasal obstruction, rhinorrhea, nasal crusting, epistaxis, nasal mass, facial/nasal pain, and anosmia (23
). On physical examination, purplish, friable nasal mucosa, mucosal
hypertrophy, nasal polyps, and yellow submucosal nodules may be observed, most commonly involving the septum and inferior turbinates (Fig. 31.3
). Thickening of sinus mucoperiosteum and radiologic paranasal opacification mark sinus involvement (24
). Of 15 patients undergoing CT with sarcoidosis and sinus symptoms, the maxillary (14
), ethmoid (9
), frontal (6
), and sphenoid (3
) sinuses demonstrated complete or partial opacification (23
Figure 31.1 Sarcoidosis. Multiple noncaseating granulomas are present along the bronchovascular interstitium. Image from Rubin E, Farber JL. Pathology, 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins, 1999.
Figure 31.2 Raised lesions of lupus pernio. (From Crapo JD, Glassroth J, Karlinsky JB, et al. Baum’s textbook of pulmonary diseases, 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2004.)
When presented with sinonasal findings in suspected sarcoidosis, histological biopsy revealing noncaseating granulomas is the definitive mode of diagnosis. With the differential diagnosis including fungal and mycobacterial infections, the respective histopathology and tissue cultures are required to rule these out. However, patients who present with Lofgren syndrome (erythema nodosum, hilar adenopathy, and polyarthralgias) can be clinically diagnosed without the necessity of biopsy (19
). ACE levels and serum calcium levels may be elevated. Though nonspecific, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) levels are often elevated. Equally important are ruling out other potential disease processes and assessing the extent of pathology, for example, antineutrophil cytoplasm antibodies (ANCA) should be measured to rule out WG.
Figure 31.3 Endoscopic view of nasal sarcoidosis. The septal mucosa, as well as the lateral nasal wall reveals nodular, erythematous swellings.
Figure 31.4 Saddle-nose deformity in WG, bloody crusts in WG, subglottic stenosis in WG. (From Koopman WJ, Moreland LW. Arthritis and allied conditions a textbook of rheumatology, 15th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2005).
Treatment should be tailored to the severity and extent of disease involvement. Many patients diagnosed with sarcoidosis do not require treatment. In symptomatic patients, nasal corticosteroids for skin disease and non-steroidal anti-inflammatory drugs (NSAIDs) may be utilized for mild disease, where higher dose systemic corticosteroids and immunomodulators for more extensive disease may be initiated with close medical follow-up. Six to nine months of prednisone, initiated at a dose of 30 to 40 mg daily followed by a 5 mg/day taper to a 10 to 20 mg/day maintenance dose, is recommended (19
). Methotrexate has been shown to be an effective therapeutic and allows for decreased doses of systemic steroid administration in a randomized double-blind trial (28
). Additionally, hydroxychloroquine, azathioprine, pentoxifylline, statins, and tumor necrosis factor (TNF-α) TNF-alpha binding proteins/inhibitors, such as infliximab, etanercept, and adalimumab, have been described for the same intent (29
). A multicenter randomized, double-blind, placebo controlled trial of infliximab has shown improvements in pulmonary function with pulmonary sarcoidosis, though the remaining biologics mentioned have otherwise not shown dramatic efficacy to date (19
). Sinonasal involvement has been shown to be a poor prognostic indicator with a high frequency of recalcitrant disease involving multiple vital organs, thus oftentimes necessitating systemic therapy (23
The goal for surgical intervention in sarcoidosis is not eradication of disease, but potential quality of life improvement. Kay and Har-El examined the role for endoscopic sinus surgery in sarcoidosis. Six of 86 sarcoidosis patients underwent endoscopic sinus surgery with indications including granulomatous or polypoid lesions causing severe nasal obstruction or blocking the osteomeatal complex leading to symptomatic chronic or acute recurrent sinusitis. CO2
laser surgery has also been described for treating such granulomas (29
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