Molecular targeted therapies for microscopic polyangiitis and granulomatosis with polyangiitis

Harigai, Masayoshi; Tsutsumino, Michi; Takada, Hideto; Nagasaka, Kenji

doi:10.3904/kjim.2018.366

Cited by 6 publications

(6 citation statements)

References 39 publications

(41 reference statements)

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“…The mainstay of the treatment in MPA is immunosuppression including glucocorticoids and cyclophosphamide. In this study, all patients were under cyclophosphamide treatment and plasma exchange was required in about 30% of the patients [14].…”

Section: Discussionmentioning

confidence: 99%

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

2020

Signa Vitae

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Background and Aim: Microscopic polyangiitis (MPA) is a rare necrotizing vasculitis that involves predominantly the small-caliber blood vessels. In this retrospective study, we aimed to evaluate the clinical findings, prognostic factors, and outcomes of MPA patients followed in two different centers. Material and Method: In this retrospective study, a total of 38 patients diagnosed and followed with MPA in two different centers between January 2005 and December 2019 were included. Medical records of the patients were reviewed retrospectively. The diagnoses were based on the definition of MPA in the Chapel Hill consensus conference (CHCC-2012). In addition to the socio-demographic characteristics, clinical features, laboratory and immunological parameters; time passed for the diagnosis, treatment, outcomes, and follow-up time were recorded for each patient. The Five Factor Scores (FFS; Revised FFS 2009) and Birmingham Vasculitis Activity Score (BVAS; Version 3) were applied in all patients. Results: In a total of 38 patients (10 females, 28 male) were included in the study. The mean age of the participants was 54.52 ± 8.21 (range: 32-74) years. The mean time passed for the diagnosis was 2.93 ± 2.03 (0.50-11) months and the mean follow-up period was 55.0 ± 30.51 (2-124) months. At admission, the mean BVAS was 18.28 ± 5.73 (8-35). The FFS results were as follows; 0 in 4 (10.5%) patients, 1 in 27 (71.1%) patients, 2 in 7 (18.4%) patients. The most common symptoms at admission were dyspnea, cough, and hemoptysis. Renal failure was present in 31 (81.5%) of the patients, at admission. The most common immunologic finding was the p-ANCA positivity in 26 (68.4%) patients. Among all patients, 25 (65.8 %) did not have any other attacks after the diagnosis. In follow-ups, 12 (31.5%) patients were passed away and vasculitis associated mortality rate was 18.4%. The effects of some factors on mortality including age ≥ 65 years, male gender, renal involvement, p-ANCA positivity, decreased oxygen saturation (< 90%), having anemia at admission, having FEV1/FVC < 70 in spirometry, having a FFS score of 2, and having a BVAS > 18 were examined (Table 6). Only having an FFS score of 2 and BVAS > 18 were determined as the factors significantly affecting mortality (P: 0.01). Conclusion: Pulmonary symptoms are more predominant in MPA. Though many patients did not have any other attacks after the diagnosis, the vasculitis associated mortality may reach about 20%. BVAS and FFS are still the best determinants of mortality.

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Section: Discussionmentioning

confidence: 99%

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

2020

Signa Vitae

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“…Due to the adverse effects of cyclophosphamide and rituximab, drugs with a novel mechanism of action in treating GPA are under trial. These include the B-cell activating factor, C5a receptor, and interleukin- 6 [59 ▪ ].…”

Section: Treatment Of Granulomatosis With Polyangiitismentioning

confidence: 99%

Current concepts in granulomatosis poly angiitis and the eye

Sivaraman

Majumder

Mahendradas

et al. 2022

Current Opinion in Ophthalmology

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Purpose of reviewGranulomatosis with polyangiitis (GPA), a multisystem disease with diverse systemic and protean ocular manifestations. Its pathophysiology, spectrum of the ocular manifestations, changing paradigms in the diagnosis of the disease, recent updates in the treatment patterns are clinically relevant to the treating clinician. They are described in this review.Recent findingsThe ocular manifestations are not included in the updated diagnostic criteria of GPA. Hence, high index of suspicion is required when a clinician encounters eye as the first clinical presentation.The concentration of interleukin -32 and 6 are correlated to disease activity of GPA.Enzyme-linked immunoassay for antineutophilic antibodies are recommended over indirect immunofluorescence to detect the subtypes with clinical correlation. The false positivity of test results in tuberculosis needs to be ruled out.Both rituximab and cyclophosphamide are equally considered as a first line of therapy of GPA in the induction phase as per randomized studies. During the continuation phase, switch over to methotrexate and mycophenolate can be considered.SummaryThe changing trends in the diagnosis, treatment can be adapted to real time clinical practice to provide the best quality of life to patients with GPA.

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“…Case reports of successful treatment with Tocilizumab have been published in literature ( 67 , 68 ). Clinical trial with TCZ and AAV is currently being considered ( 69 ).…”

Section: Emerging Therapy In Anca Vasculitismentioning

confidence: 99%

Primary Vasculitis in Childhood: GPA and MPA in Childhood

Jariwala

Laxer

2018

Front. Pediatr.

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Childhood onset anti-neutrophilic cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare group of primary systemic vasculitides affecting medium and small blood vessels. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal limited ANCA vasculitis. These disorders are associated with severe clinical manifestations, frequent relapses and a high cumulative morbidity, and often present with multisystem involvement. Renal involvement is common in the pediatric age group, characterized by pauci-immune necrotizing and crescentic glomerulonephritis which frequently progresses to chronic kidney disease in adulthood. ANCAs against proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) remain the hallmark of AAV and are integral to the disease pathogenesis. Newer understanding of neutrophil extracellular traps and complement activation have provided better insights into disease pathogenesis. A pediatric vasculitis working group has developed and validated childhood vasculitis classification criteria and disease activity and damage scores. No specific pediatric treatment recommendations exist due to rare nature of the illness in pediatric population. Smaller case series have been published on the efficacy of adult treatment regimens in pediatric patients. The prognosis often remains guarded with frequent relapses and a high cumulative morbidity. The aim of this article is to provide a comprehensive review on pediatric AAV with a focus on recent observations regarding epidemiology, disease pathogenesis, treatment, and prognosis.

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Molecular targeted therapies for microscopic polyangiitis and granulomatosis with polyangiitis

Cited by 6 publications

References 39 publications

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

Current concepts in granulomatosis poly angiitis and the eye

Primary Vasculitis in Childhood: GPA and MPA in Childhood

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