Rare presentation of granulomatosis with polyangiitis.

Authors :: Hines A
Bello V
Iftikhar A
Zein H
Source :: BMJ case reports [BMJ Case Rep] 2019 Feb 22; Vol. 12 (2). Date of Electronic Publication: 2019 Feb 22.
Publication Year :: 2019
Abstract: A 62-year-old man with no pertinent medical history presented with lower extremity weakness and worsening distal fingertips and toe cyanosis/gangrene. In the outpatient setting, he was initially being treated for Raynaud's phenomenon with a calcium channel blocker. On presentation, the patient had elevated inflammatory markers and white blood cell count. Serum vasculitis panel (proteinase-3 antibody) supported the diagnosis of granulomatosis with polyangiitis. His hospital course was complicated by ischaemic stroke and a diagnosis of mononeuritis multiplex in his lower extremities. After initiating therapy with intravenous steroid and rituximab, his symptoms overall improved including cyanotic fingertips/toes. His inflammatory markers and leucocytosis also improved. Outpatient follow-up consisted of further rituximab infusions and unrelated umbilical hernia incarceration which required surgery. He was found incidentally to have subsegmental pulmonary emboli which most likely occurred during the initial presentation prior to his diagnosis. The patient moved out of state and was lost to follow-up.<br />Competing Interests: Competing interests: None declared.<br /> (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)