Locations:
Search IconSearch

Case Report: Granulomatosis with Polyangiitis and Persistent Shortness of Breath

Multidisciplinary management resolves complex case

21-RHE-2519425-GranulomatosisPolyangiitis-PersistentShortnessOfBreath-CQD_650x450

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.

Advertisement

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

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.

A Case of Persistent Shortness of Breath in Granulomatosis With Polyangiitis (GPA)

“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.

Advertisement

Related Articles

patient consult
Key Elements for Optimizing Care in People with Vasculitis

Evidence-based therapies, monitoring, prevention and more

RCVL-S
Case: When Central Nervous System Vasculitis Is Suspected

Holistic approach is necessary to ensure a correct diagnosis

21-RHE-2528446-Monitoring-patients-ANCA-associated-vasculitis-CQD_650x450
Keys for Recognizing Drug-Induced Vasculitis

Knowing the affected organs and vessels can help in identifying cause

pulmonary artery stenosis
Takayasu Arteritis Case Study: Pulmonary Embolism or Something Else?

When to consider the possibility of pulmonary artery involvement

21-RHE-2528447-case-extracranial-Giant-cell-arteritis-CQD_650x450
A Challenging Case of Giant Cell Arteritis

When GCA initially presents without cranial symptoms

21-RHE-2528446-Monitoring-patients-ANCA-associated-vasculitis-CQD_650x450
Case Report: Long-Term Monitoring a Must for ANCA-Associated Vasculitis

Relapses are frequent even with long periods of remission

A series of brain MRI images used to help diagnose CNS vasculitis
Neurologic Involvement in ANCA-Associated Vasculitis

Raising awareness of a common manifestation

18-HRT-5549-thoracicAortAneur-650×450
Treatment of Chronic and Relapsing Takayasu’s Arteritis

Prevention of progression and complications is the primary goal

Ad