The clinicopathologic characteristics of granulomatosis with polyangiitis (Wegener’s): a retrospective study of 45 patients in Korea

Abstract

Taken together, in Korean patients with GPA, the granulomatous form was predominant and associated with a younger age at diagnosis and a lower frequency of renal involvement than the vasculitic form. ANCA positivity was found in 68.9 % and associated with renal involvement and higher mortality.

Myeloperoxidase–Antineutrophil Cytoplasmic Antibody (ANCA)–Positive Granulomatosis With Polyangiitis (Wegener's) Is a Clinically Distinct Subset of ANCA‐Associated Vasculitis: A Retrospective Analysis of 315 Patients From a German Vasculitis Referral Center

“…MPO‐ANCAs are found in 2.6–9.0% of mainly European patient populations with GPA . To our knowledge, only small cohorts of patients with MPO‐ANCA–positive GPA have been described to date , mainly from eastern Asia, where MPO‐positive AAV (especially MPA) is more frequent than in northern Europe and a higher proportion of GPA patients have MPO‐ANCA . Based on clinical observations, we hypothesized that patients with MPO‐ANCA–positive GPA might form a clinically distinct subset of AAV different from both patients with PR3‐ANCA–positive GPA and those with MPO‐ANCA–positive MPA.…”

Limited granulomatosis with polyangiitis in an adolescent with Crohn's disease on infliximab therapy: cause or coincidence?

“…Our patient's presentation fits with the description of 'limited' form of GPA, which is known to be localised to the lungs, is more common in females, has a younger age of onset, and is less likely to be associated with ANCA positivity (26). ANCA negative GPA is generally associated with lower incidence of renal involvement and better long-term survival (27). ANCA positivity is not an absolute requirement for the diagnosis of GPA as per the American College of Rheumatology criteria (28) or in the updated definition provided by the Chapel Hill Consensus Conference (29).…”

Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Korea: A Narrative Review

“…In terms of GPA, Kim, et al39 reported concrete data regarding clinicopathologic characteristics of Korean patients with GPA. Besides Koran patients with MPA, initial ANCA positivity was significantly associated with renal involvement of GPA and all-cause mortality during the follow-up.…”

“…The second study reported that 1-year- and 3-year-cumulative survival rates were 93.9% and 89.2% in MPA patients 35. The third study reported that 22.2% of GPA patients died during follow-up 39. In 2016, Lee, et al46 investigated long term prognosis in 48 Korean patients with renal vasculitis.…”

“…However, most clinical studies regarding serious infection have been conducted in Western countries and only a few observational studies have been undertaken in Korea. Kim, et al39 reported 20 of 45 Korean patients with GPA (44.4%) suffered from various infections, including pneumonia, bacteraemia, septic shock, peritonitis, and so on. Yoo, et al56 reported retrospective data on hospitalised infections in 154 Korean patients with AAV.…”