A Patient with Granulomatosus with Polyangiitis (Wegener’s Granulomatosis): ACase Report

Abstract

Background: Granulomatosus with Polyangiitis (GPA) is an Antineutrophilic

Cytoplasmic Antibodies (ANCA) Associated Vasculitis (AAV)
which involves small to moderate-sized vessels. GPA has a variety of clinical
manifestations caused by tissue ischemia and organ affected. Diagnosis of
GPA remains challenging, and its actual incidence may be higher than
reported. In 1990, the American College of Rheumatology (ACR) published
diagnostic criteria for GPA. Guidelines of management of GPA has improved
survival in last decade, but results remain unsatisfactory. Induction agents
with immunosuppressive agents and glucocorticoid, and the newer agent
Rituximab are recommended as first choice treatment. Case presentation:
A 64-year-old male presented with current episode of joint pain and a history
of recurrent respiratory tract infections. After a series of laboratory and
radiologic examinations, lung biopsy was performed, and the result was
suggestive of GPA. The patient was managed with induction remission agent
and reported improvement in both clinical and laboratory parameters.
Conclusion: Granulomatosus with Polyangiitis is a limitedly reported case.
This report was presented to raise awareness of the diagnosis when faced
with similar clinical symptoms. Early detection and diagnosis in GPA allow
for prompt and better management with the target to achieve and maintain
remission, as demonstrated in this case.