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http://dx.doi.org/10.4103/lungindia.lungindia_263_22DOI Listing

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: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvement is exceptional and it represents a challenge that requires a rapid intervention with the potential of displaying a fulminant evolution. : We report a 64-year-old male with an 18-year history of GPA who presented atypical low back pain.

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotising vasculitis affecting small vessels accompanied by eosinophilic inflammation. Biological therapies, particularly anti-interleukin-5 (IL-5) monoclonal antibodies, have been shown to be effective in treating refractory EGPA. Mepolizumab, an anti-IL-5 monoclonal antibody, has been approved in Japan for the treatment of EGPA and has a significant glucocorticoid-sparing effect.

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Objective: The ADVOCATE trial demonstrated that treatment of active granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) with avacopan was noninferior in achieving remission at week 26 and superior for sustained remission at week 52 compared with a prednisone taper. This analysis of ADVOCATE evaluated the efficacy and safety of avacopan in patients with ear, nose, throat (ENT), or lung manifestations.

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Objective: This study investigated the effects of early treatment and pathophysiology on eosinophilic granulomatosis with polyangiitis neuropathy (EGPA-N).

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We report the case of a 75-year-old woman who presented with fever, right back pain, paresthesia in the right extremities, erythema, purpura, and nodules. She had previously initiated dialysis due to rapidly progressive glomerulonephritis and was transferred to our hospital. Imaging studies revealed multiple cerebral and splenic infarcts and hemorrhage encapsulating the right kidney, likely due to microaneurysms in multiple renal arteries.

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