Case Report: Granulomatosis with Polyangiitis and Persistent Shortness of Breath

A 38-year-old male presented to an outside hospital with dry cough and shortness of breath, inflammatory polyarthritis, purpuric rash, chronic sinus congestion and pain, and ear pain and hearing loss. Computed tomography of his sinuses showed pansinusitis, and an otorhinolaryngology exam revealed otitis media. Audiologic testing demonstrated mixed hearing loss, and his laboratory tests showed elevated C-reactive protein and sedimentation rate as well as the presence of proteinase 3 anti-neutrophil cytoplasmic antibody.

Computed tomography (CT) of the chest was normal, and a biopsy of his rash revealed leukocytoclastic vasculitis. He was diagnosed with granulomatosis with polyangiitis (GPA) affecting his sinuses, ears, skin and joints.

GPA is an antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitide disorder, characterized by systemic autoimmune small-vessel vasculitis. It can affect the lungs, kidneys, airway and sinonasal regions. Despite many advances in treatment, 50 to 70 percent of patients experience relapses after remission induction.

The patient was placed on induction therapy of cyclophosphamide and prednisone, which resolved all of his symptoms except for his shortness of breath.

He sought a second opinion at Cleveland Clinic’s Orthopaedic and Rheumatologic Institute, where he underwent several more tests, including an echocardiogram, pulmonary function test, broncoscophy and chest CT. Watch to find out from Alexandra Villa-Forte, MD, MPH, staff in the Center for Vasculitis Care and Research in Cleveland Clinic’s Department of Rheumatic and Immunologic Diseases. what physicians found and how the patient’s shortness of breath was completely resolved.

“The key point in this case is not to forget that subglottic and bronchial stenoses are important causes of persistent shortness of breath and cough in GPA patients despite systemic therapy, and that therapy with local corticosteroid injection and dilation is usually necessary in addition to systemic therapy,” says Dr. Villa-Forte.