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 and C-reactive protein), 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. Patients may also require concurrent systemic therapy.
- Severe nasal crusting with friable underlying mucosa, septal perforation, and saddle nose deformity.
- ANCA (antineutrophil cytoplasmic autoantibodies), chest X-ray, and urinalysis.
- Nasal biopsy of suspicious lesions; possible renal biopsy.
Wegener's granulomatosis is an idiopathic vasculitic and autoimmune process that typically involves the upper and lower respiratory tracts. It occurs in all age groups and predominantly affects white populations. The spectrum of disease presentation ranges from localized to disseminated forms, but the majority of patients have otolaryngologic manifestations. Classically, Wegener's granulomatosis involves a triad of necrotizing granulomas of the upper and lower airways, glomerulonephritis, and disseminated vasculitis. 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.
The more limited, localized form of Wegener's granulomatosis 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 found with progressive disease, and can lead to saddle-nose deformity due to loss of cartilaginous support and a resultant a dorsal concavity. Other potentially involved head and neck sites are (1) the orbit, with nasolacrimal duct obstruction, orbital pseudotumor, episcleritis, or peripheral ulcerative keratitis; (2) the ear, including serous otitis media, with or without mastoiditis, and possible sensorineural hearing ...