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GRANULOMATOUS AND AUTOIMMUNE DISEASES

General Considerations

Granulomatous and autoimmune diseases are characterized by a systemic chronic inflammatory process and a predilection for particular organ systems. Occasionally, patients may present with florid nasal symptoms such as severe crusting, inflammation, and saddle nose deformity, which raise suspicion early in the evaluation. However, sinonasal manifestations are often nonspecific, including nasal obstruction, rhinorrhea, and recurrent sinusitis. The practitioner must therefore include these diseases in the differential diagnosis of chronic sinonasal symptoms and assess for systemic manifestations in the history and physical examination.

Although obtaining biopsies of suspicious lesions is critical for establishing a diagnosis, specimens often demonstrate nonspecific chronic inflammation and necrosis. Helpful adjunctive tests include inflammatory markers (erythrocyte sedimentation rate [ESR] and C-reactive protein [CRP]), complete blood cell count, various autoimmune serologies, chest x-ray, urinalysis, and bacterial and fungal cultures.

The goals of managing the sinonasal manifestations of these diseases are to provide symptomatic relief of nasal obstruction and crusting and to reduce the incidence and severity of secondary sinusitis from ostial obstruction by reducing mucosal edema and facilitating mucociliary clearance. While surgical intervention plays a role in establishing the diagnosis and providing symptomatic relief, treatment of the underlying condition typically requires concurrent systemic therapy.

GRANULOMATOSIS WITH POLYANGIITIS (WEGENER GRANULOMATOSIS)

ESSENTIALS OF DIAGNOSIS

  • Severe nasal crusting with friable underlying mucosa, septal perforation, and saddle nose deformity.

  • Cytoplasmic antineutrophil cytoplasmic autoantibodies (c-ANCA), chest x-ray, and urinalysis.

  • Nasal biopsy of suspicious lesions; possible renal biopsy.

General Considerations

Granulomatosis with polyangiitis, previously known as Wegener granulomatosis, is an idiopathic vasculitis of medium and small vessels that is characterized by necrotizing granulomatous inflammation. It occurs in all age groups, but the most common age of presentation is the sixth and seventh decade of life. It predominantly affects white populations, and men and women are similarly affected. Classically, granulomatosis with polyangiitis involves a triad of necrotizing granulomas of the upper and lower airways, glomerulonephritis, and disseminated vasculitis. The spectrum of disease presentation ranges from localized to systemic forms, but the majority of patients have otolaryngologic manifestations. Upper respiratory tract symptoms may occur in up to 90% of patients, and sinonasal symptoms may be the only systemic manifestation in 30%. The previously undiagnosed patient may present with a pattern of chronic or recurrent sinusitis, managed medically and often surgically with variable clinical improvement.

Clinical Findings

A. Symptoms and Signs

The more limited, localized form of granulomatosis with polyangiitis typically presents with a several-week history of upper respiratory infection symptoms that are unresponsive to standard medical treatment, with the presence of serosanguineous nasal drainage and characteristic pain over the dorsum. Nasal exam is notable for significant bilateral nasal crusting with underlying friable mucosa, particularly over the nasal turbinates, with possible extension to the nasopharynx. Septal perforations may be ...

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