A 36-Year-Old Woman With a Worsening Cough and Hearing Loss

Nicole Davey-Ranasinghe, MD; Constantine K. Saadeh, MD

Disclosures

September 05, 2019

Discussion

Eosinophilic granulomatosis with polyangiitis (EGPA)—formerly termed "Churg-Strauss syndrome"—is a vasculitis of small and medium vessels. The vasculitis itself is not always clinically apparent; this is especially true early in the disease. The disease usually progresses from a prodromal phase to an eosinophilic phase and then to a vasculitic phase. The disease is multisystem and is characterized by asthma, chronic rhinosinusitis, and peripheral eosinophilia.[1]

The lungs, upper airways, and skin are the most commonly involved organs. However, any organ system can be involved, including the cardiovascular, neurologic, renal, gastrointestinal, and musculoskeletal systems. Eosinophilic lymphadenopathy has also been noted in patients with EGPA. As with other vasculitides, the risk for thromboembolic events is increased in patients with EGPA.

Asthma is the primary feature of EGPA and is present in the vast majority of patients. The asthma generally precedes the vasculitis and can be present for a decade or longer before the vasculitis is clinically evident. After onset of vasculitis, asthma exacerbations often increase in both frequency and severity. Patients are often taking inhaled or systemic glucocorticoids for underlying asthma; thus, the vasculitis does not become apparent until the glucocorticoids are weaned or discontinued.

Upper-airway and ear disease—including ear, nose, and throat involvement, with nasal polyposis—is present in most patients. Sensorineural hearing loss can also occur. Compared with patients with granulomatosis with polyangiitis (GPA), those with EGPA lack necrotizing lesions of the nasopharynx. Cutaneous vasculitis can appear at any time during the course of the disease. Cutaneous granulomas present as tender, subcutaneous nodules on the extensor surfaces.

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