Dual myeloperoxidase-antineutrophil cytoplasmic antibody- and antiglomerular basement membrane antibody-positive cases associated with prior pulmonary fibrosis: a report of four cases

Abstract

Four cases that showed prior pulmonary fibrosis as well as subsequent RPGN and pulmonary hemorrhage were both MPO-ANCA- and anti-GBM Ab-positive at the time of RPGN. The glomeruli disclosed features compatible with anti-GBM Ab disease, but the clinical and pathological vasculitic manifestations, including prior pulmonary fibrosis that might be an early manifestation of ANCA disease, suggested the occurrence of MPO-ANCA-associated vasculitis. Furthermore, 1 case subsequently showed repetitive pulmonary hemorrhage with re-elevated MPO-ANCA positivity but without anti-GBM Ab positivity, and this event was possibly due to MPO-ANCA-associated alveolar capillaritis. As anti-GBM Ab disease is generally thought not to manifest the clinical and pathological features of vasculitis excluding the kidney, MPO-ANCA might be a key factor regarding the occurrence of this dual positive disease.

Anti-glomerular basement membrane (anti-GBM) disease accompanied by vasculitis that was not positive for antineutrophil cytoplasmic antibodies to myeloperoxidase and proteinase 3: a report of two cases and the incidence of anti-GBM disease at one institution

“…Only a few papers have shown photographs of necrotizing angiitis [23,24,26], but these pathological findings might have been due to vasculitis which resulted from unrecognized ANCA at the time. In contrast, there are several articles which described the presence of vasculitic manifestations but did not show the pathological findings in the cases positive for both anti-GBM Ab and MPO-or PR3-ANCA [5][6][7][10][11][12][13][14]. In fact, only three recent reports of cases demonstrating anti-GBM Ab and MPO-ANCA positivity presented with pathological findings of vasculitis [8,9,31].…”

“…They consisted of a case with fibrinoid angiitis in the kidney and two cases with angiitis not accompanied by fibrinoid degeneration in the kidney or muscle, respectively. In our own institution, we experienced one additional case which demonstrated angiitis without fibrinoid degeneration and a Time interval between the initial symptoms and the admission to the affiliated hospital or our university hospital was positive for anti-GBM Ab as well as MPO-ANCA [14]. Therefore, it should be considered whether cases of Goodpasture's syndrome or anti-GBM disease associated with vasculitis are positive for MPO-ANCA, PR3-ANCA, other ANCA subsets, or IF ANCA [6-9, 11, 12, 14-16].…”

“…The former group is categorized as having anti-GBM disease with either MPO-or PR3-ANCA (Group I), while the latter group is categorized as having anti-GBM disease without MPO-and PR3-ANCA (Group II). A manuscript about Group I consisting of 4 patients was accepted for publication in the Journal of Clinical and Experimental Nephrology (in press [14]). Therefore, we studied the cases of Group II and identified two patients who demonstrated vasculitis.…”

“…In addition, cases of anti-GBM disease, which are accompanied by either myeloperoxidase (MPO)-or proteinase 3 (PR3)-anti-neutrophil cytoplasmic antibody (ANCA), have previously been described in various journals over the past 20 years including our studies [5][6][7][8][9][10][11][12][13][14]. The cases with dual antibodies are different from the cases of Goodpasture's syndrome as well as anti-GBM disease with regard to the clinical manifestations of vasculitis, patient prognosis, age of the patients, and the response to treatment [6][7][8][14][15][16]. At our institution, we experienced four patients who showed clinical features compatible with anti-GBM disease, but lacking positivity for MPO-ANCA and PR3-ANCA.…”

Microscopic polyangiitis associated with antineutrophil cytoplasmic antibodies: Clinical features

“…Нами установлено, что при МПА в исходе альвеолита возможно формирование фиброза легких, в том числе диф-фузного ИЛФ, который мы диагностировали у 5 пациентов МПА-аМПО. Опубликован ряд ретроспективных иссле-дований легочного фиброза, ассоциированного с АНЦА, которые продемонстрировали, насколько недооценена эта патология в контексте АНЦА СВ [35][36][37][38][39][40]. При АНЦА СВ отмечена связь развития ИЛФ с пожилым возрастом (70 лет), гиперпродукцией аМПО и неблагоприятным прогно-зом.…”