Granulomatosis with Polyangiitis Presenting as a Diagnostic Challenge: A Report of two Cases with Literature Review

• Main Authors: Jahnvi Dhar, Alpana Meena, Mradul Kumar Daga, Sunita Aggarwal
• Format: Article
• Language: English
• Published: JCDR Research and Publications Private Limited 2018-04-01
• Series: Journal of Clinical and Diagnostic Research
• Subjects: diffuse alveolar haemorrhage; necrotising scleritis; pulmonary renal syndrome
• Online Access: https://jcdr.net/articles/PDF/11442/34003_F(SHU)_PF1(BT_RA_MLS_AnG)_PFA(MLS_SHU)_PB(MLS_AP)_PN(AP).pdf
• ISSN: 2249-782X; 0973-709X

Vasculitis is defined as a clinicopathological entity characterised by inflammation and destruction of the wall of the blood vessel. Granulomatosis with Polyangiitis (GPA), earlier commonly known as Wegener Granulomatosis (WG) is a primary vasculitic pathology, which is a systemic disease with myriads of clinical presentations. It is an uncommon disease presenting with the triad of involvement of upper and lower respiratory tracts along with renal involvement in the form of glomerulonephritis. However, isolated organ involvement as a first clinical presentation without a chronic course of the disease culminating into a diagnosis of GPA is very uncommon and only isolated case reports are available in literature. Here, we describe two case reports of GPA, one of a young girl who presented with chest pain and suddenly developed necrotising scleritis with loss of vision and the second a middle aged female who presented with sudden onset breathlessness without haemoptysis and deranged Kidney Function Test (KFT) which diagnosed it as Pulmonary Renal Syndrome (PRS) {Diffuse Alveolar Haemorrhage (DAH)}. In both the cases, a strong clinical suspicion of vasculitis led to the diagnosis of GPA. These two case reports highlight the importance of timely intervention which resulted in a fruitful outcome for both the patients.