Rituximab in anti-GBM disease: A retrospective study of 8 patients

Touzot, Maxime; Poisson, Johanne; Faguer, Stanislas; Ribes, David; Cohen, Pascal; Geffray, L.; Anguel, Nadia; Harel, François; Karras, Alexandre; Cacoub, Patrice; Dürrbach, Antoine; Saadoun, David

doi:10.1016/j.jaut.2015.04.003

Cited by 89 publications

(68 citation statements)

References 18 publications

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“…In patients presenting with an initial requirement for dialysis, however, renal recovery occurred in only 8% at 1 year. Other reports have described similarly low levels of renal recovery in patients presenting with dialysis-dependent kidney failure, with the highest rate of approximately 20% recovery in one series (66). Predictors of poor renal outcome include severity of renal dysfunction at presentation, the proportion of glomeruli affected by crescents, and oligoanuria at presentation (17,18,74).…”

Section: Outcome and Prognosismentioning

confidence: 72%

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Anti-Glomerular Basement Membrane Disease

McAdoo¹,

Pusey²

2017

CJASN

315

303

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Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggests that environmental factors, including infection, may trigger disease in genetically susceptible individuals. The majority of patients develop widespread glomerular crescent formation, presenting with features of rapidly progressive GN, and 40%-60% will have concurrent alveolar hemorrhage. Treatment aims to rapidly remove pathogenic autoantibody, typically with the use of plasma exchange, along with steroids and cytotoxic therapy to prevent ongoing autoantibody production and tissue inflammation. Retrospective cohort studies suggest that when this combination of treatment is started early, the majority of patients will have good renal outcome, although presentation with oligoanuria, a high proportion of glomerular crescents, or kidney failure requiring dialysis augur badly for renal prognosis. Relapse and recurrent disease after kidney transplantation are both uncommon, although de novo anti-GBM disease after transplantation for Alport syndrome is a recognized phenomenon. Copresentation with other kidney diseases such as ANCAassociated vasculitis and membranous nephropathy seems to occur at a higher frequency than would be expected by chance alone, and in addition atypical presentations of anti-GBM disease are increasingly reported. These observations highlight the need for future work to further delineate the immunopathogenic mechanisms of anti-GBM disease, and how to better refine and improve treatments, particularly for patients presenting with adverse prognostic factors.

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Section: Outcome and Prognosismentioning

confidence: 72%

“…There are several reports of rituximab use, as either "add-on" to standard therapy or as a substitute for cyclophosphamide in patients who are intolerant (66). Similarly, the use of mycophenolate mofetil and cyclosporine has been reported in individual cases or small series (67-69).…”

Section: Treatmentmentioning

confidence: 99%

Anti-Glomerular Basement Membrane Disease

McAdoo¹,

Pusey²

2017

CJASN

315

303

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“…The duration is usually for 2 weeks at least and is modulated according to the clinical status and the level of circulating autoantibodies [85,86]. Rituximab has been used successfully in severe acute as well as in refractory or relapsing disease [87,88].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.

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“…However, the immunosuppressive regimens are highly heterogeneous among studies on GS and, to date, no randomized study has defined the best regimen. While the standard treatment ultimately results in serological disappearance of anti-GBM autoAb in most patients, the addition of anti-CD20 rituximab monoclonal antibody therapy (375 mg/m 2 /week for 4 weeks) has been proposed for the extremely unusual patients with refractory or relapsing GS, and for patients with CYC intolerance [6]. Combined studies on GS suggest that the two main causes of death remain the development of intractable acute respiratory distress syndrome related to severe pulmonary hemorrhage, or an immunosuppression-related severe bacterial infection.…”

Section: Introductionmentioning

confidence: 99%