Authors: Gill KL, Deshmukh P, Snyder T, Kaushik P.
Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis, is a rare small vessel vasculitis that is a part of the ANCA-associated family. First distinctly identified by Fredrick Wegener in 1936, GPA has had little light shed on the direct pathophysiology for the development of the disease. Classically the disease presents in Caucasian males during the fifth to seventh decades of life [1]. GPA can present with a wide array of symptoms and therefore the diagnosis can be easily missed. Unfortunately, without proper prompt treatment the disease can progress causing irreversible organ damage leading to chronic, relapsing dysfunction and possible death. Given this, early recognition and treatment of the disease is essential. This becomes difficult when given an atypical patient presentation compounded with nondescript symptoms. In this case-presentation, we review an atypical presentation of GPA from a rural rheumatology clinic in the United States with the intention of expanding the current limited literature. Through thorough literature-search we were able to find reports of a 12 year old female that was diagnosed with GPA, and a young 8 -year old American Indian/Native American (AI/NA) female diagnosed with microscopic polyangiitis, however, to our knowledge this is the youngest known AI/NA patient to be diagnosed with GPA at 13 years of age [2,3].
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