Citation

Anthony AIL, Ibrahim ZA, Li-Cher L (2019) From Triumph to Tribulation: A Granulomatous Polyangiitis Case Report. Int J Respir Pulm Med 6:113. doi.org/10.23937/2378-3516/1410113

Copyright

© 2019 Anthony AIL, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

CASE REPORT | OPEN ACCESSDOI: 10.23937/2378-3516/1410113

From Triumph to Tribulation: A Granulomatous Polyangiitis Case Report

Albert Iruthiaraj Lourdesamy Anthony1*, Zainal Abidin Ibrahim2 and Li-Cher Loh3

1Respiratory Unit, Taiping Hospital, Ministry of Health Malaysia, Malaysia

2Ramsay Sime Darby Diagnostic Laboratory, Georgetown, Penang, Malaysia

3RCSI & UCD Malaysia Campus, Georgetown, Penang, Malaysia

Abstract

Background

Granulomatous Polyangiitis (GPA) is a rare form of small vessel vasculitis characterized by the demonstration of necrotizing granulomatous inflammation histologically.

Case

We report the case of a 62-years-old woman with symptoms of cough and significant loss of weight with radiological findings to support a left lower lobe lung mass. The patient was initially suspected to have metastatic lung malignancy until biopsy results suggested necrotizing granulomatous inflammation. A diagnosis of limited GPA was made when the cytoplasmic antineutrophil cytoplasmic antibodies, (c-ANCA) and antiproteinase-3 antibodies (anti-PR3) was elevated. The patient was treated with combination therapy of prenisolone (1 mg/kg) and methotrexate which induced clinical remission within weeks and radiological complete remission within 3 months.

However, as prednisolone was tapered down, there were suggestions of a relapse. Conclusion: The presentation of GPA can be highly variable and pose a diagnostic challenge to clinicians. In cases at risk for relapse, treatment should be continued for at least 18-24 months.