Case of EGPA and Eosinophilic Chronic Rhinosinusitis Concomitant with IgG4 Related Disease

Abstract

Eosinophilic granulomatosis with polyangiitis EGPA is a systemic vasculitis in patients with bronchial asthma and eosinophilic rhinosinusitis. Serum IgG4 levels are markedly elevated in patients with active EGPA, a disease which has been closely associated with IgG4 related disease IgG4RD. A 68 year old male with a history of asthma and eosinophilic rhinosinusitis developed vasculitis and orbital symptoms. The results of a laboratory examination showed eosinophilia 4,067/μl ; 39% , while image evaluations revealed hypertrophy of the rectus muscles, trigeminal nerve, lachrymal gland, and bilateral submandibular glands. Biopsy of the paranasal sinus showed the prominent infiltration of eosinophils and IgG4 positive plasma cells. The patient was diagnosed with EGPA concomitant with IgG4RD and treated with systemic steroids. Although concomitant cases of EGPA with IgG4RD are extremely rare, clinical manifestations associated with both diseases are sometimes mixed. Therefore, systemic scrutiny may be required for cases of EGPA with high serum IgG4 levels and pathognomonic symptoms or findings of IgG4RD.

A Case of Eosinophilic Granulomatosis with Polyangiitis Complicated with A IgG4 Related Disease Like Symptoms

“…There are some reports about CSS concomitant with clinical conditions similar to IgG4-RD [7, 8]. However, reports about the orbital manifestations similar to IgG4-RD in EGPA were relatively rare [9].…”

Clinical and immunological variants of ANCA-associated systemic vasculitis: a look at the phenomenon of IgG4 overproduction. Literature review and own observations

“…Характерные для IgG4-СЗ проявления встречались при всех нозологических формах АНЦА-СВ, частота гиперпродукции аПР3 и аМПО не различалась. Имеется описание IgG4-СЗ у пациента с АНЦА-негативным ГПА [30] при ЭГПА [31]. В ряде случаев характерные для IgG4-СЗ проявления при АНЦА-СВ не сопровождались повышением сывороточной концентрации IgG4 или отсутствовали типичные гистологические критерии IgG4-СЗ.…”