A Rare Case of Pulmonary-Renal Syndrome With Triple-Seropositive for Myeloperoxidase-Anti-Neutrophil Cytoplasm Antibody (MPO-ANCA), Proteinase 3 (PR3)-ANCA and Anti-Glomerular Basement Membrane (GBM) Antibodies

Abstract

Anti-glomerular basement membrane (anti-GBM) disease and antineutrophil cytoplasm antibody (ANCA)-associated vasculitis are the main causes of pulmonary-renal syndrome (PRS). The concurrence of both ANCA -myeloperoxidase (MPO) and proteinase 3 (PR3) -and anti-GBM antibodies has been described, although positivity for all three antibodies has rarely been reported. The natural history of triplepositive patients as well as the best therapeutic approach remains unknown. We describe a case of an 80-year-old woman that presented to the emergency department with a 3-month history of progressive fatigue, malaise and anorexia, and 5 weeks of cough with bloodstreaked sputum and progressive peripheral edema. Through the complementary study, a rare diagnosis of PRS with triple-seropositive for both ANCA (MPO and PR3) and anti-GBM antibodies was made in a patient with untreated chronic hepatitis B virus infection. She was treated with glucocorticoid, cyclophosphamide, plasma exchange and entecavir, with pulmonary recovery. Renal function did not improve. After 2 years, the patient is still in dialysis, but did not have relapse of alveolar hemorrhage and ANCA and anti-GBM antibody titers remain negative. The authors intend to warn to PRS, in particular this rare cause, since delaying diagnosis can lead to significant morbidity and mortality for patients.

A Fatal Case of Rapidly Progressive Glomerulonephritis With Two Anti-neutrophil Cytoplasmic Antibodies and Anti-glomerular Basement Membrane Antibody: A Description of Autopsy Findings

“…Crescentic glomerulonephritis conditions, such as antineutrophil cytoplasmic antibody (ANCA)-associated nephritis and anti-glomerular basement membrane (GBM) disease, are typical examples of RPGN. Single ANCA-positive and double seropositive cases are the most common; however, triple seropositive RPGN cases with myeloperoxidase-ANCA (MPO-ANCA), proteinase-3-ANCA (PR3-ANCA), and anti-glomerular basement membrane (GBM) antibodies have also been reported [ 1 , 2 ]. However, the number of case reports is small, and there is no consensus regarding the pathology and treatment of triple-seropositive RPGN.…”

Triple Jeopardy: Rapidly Progressive Glomerulonephritis Induced by Triple Seropositive Disease—A Rare Case

“…DPD is a rare disease that was first coined in the 1980s by Omotoso et al [ 2 ]. About 20–40% of anti-GBM diseases are equally ANCA positive and 5–14% vice versa [ 3 , 4 ]. A “triple positive” disease (anti-GBM, myeloperoxidase (MPO), and proteinase 3 (PR3)) causing an isolated renal failure is even rarer [ 3 ].…”

“…About 20–40% of anti-GBM diseases are equally ANCA positive and 5–14% vice versa [ 3 , 4 ]. A “triple positive” disease (anti-GBM, myeloperoxidase (MPO), and proteinase 3 (PR3)) causing an isolated renal failure is even rarer [ 3 ]. Due to its rarity, its natural history, prognosis, and appropriate management remain unknown [ 3 ].…”

“…A “triple positive” disease (anti-GBM, myeloperoxidase (MPO), and proteinase 3 (PR3)) causing an isolated renal failure is even rarer [ 3 ]. Due to its rarity, its natural history, prognosis, and appropriate management remain unknown [ 3 ]. We present a case of a 62-year-old Caucasian male who presented to the emergency department with dyspnea on exertion for about a month.…”

Pulmonary Renal Syndrome Caused by Anti-Glomerular Basement Membrane Disease in Combination With Anca- Associated Vasculitis After Sars-Cov-2 Infection

“…7, № 3 менно с двумя типами АНЦА -антимиелопероксидаза и анти-протеиназа-3 [11]. По данным разных авторов, 10-40 % анти-ГБМ положительных пациентов также имеют положительные АНЦА, а у 5-14 % АНЦА-поло жи тельных пациентов выявляют анти-GBM антитела [8,10,[12][13][14]. Этот так называемый двойной антитело-положительный фенотип нередко обусловливает атипичную клиническую картину, что приводит к задержке установления правильного диагноза, связан с плохими исходами -частыми обострениями и высокой смертностью [9,11,15].…”