musculoskeletal and connective tissue disorders
Granulomatosis with Polyangiitis (GPA)
Treatment of GPA
To induce remission in life- or organ-threatening GPA, high-dose corticosteroids plus eithercyclophosphamideorrituximab To induce remission in less severe GPA, corticosteroids and eithermethotrexateorrituximab To maintain remission,rituximabalone or another drug such asmethotrexate,azathioprine, ormycophenolatemofetil (rituximabplus another of these drugs, sometimes with a low dose of a corticosteroid, if patients have multiple relapses or GPA is difficult to control) Kidney transplantation if necessary Treatment of granulomatosis with polyangiitis depends on the severity of disease. A multidisciplinary approach is required for multiorgan disease, often including a rheumatologist, otorhinolaryngologist, pulmonologist, and nephrologist. (see Induction of remission ). Efficacies of ( Treatment references ). Whether For less severe disease, corticosteroids andmethotrexateare used to induce remission.Rituximabmay be used instead ofmethotrexate. For upper respiratory tract manifestations,rituximabappears to maintain remission better thancyclophosphamide,methotrexate, orazathioprine. Corticosteroids are tapered to as low a dose as possible or discontinued. Irrigation of sinuses with saline, with or withoutmupirocin2% nasal ointment, helps minimize crusting and secondary staphylococcal infections. Treatment of subglottic stenosis is difficult. Systemic immunosuppressants may not be effective. Intralesional injection of long-acting corticosteroids, with gentle progressive dilation, markedly improves outcome and limits the need for tracheostomy. Patients should be taught about the disorder so that relapses can be detected early. Kidney transplantation has been successful; the risk of relapse after transplantation is reduced compared with maintenance dialysis treatment (possibly due in part to use of immunosuppressants to prevent rejection). 1.Stone JH, Merkel PA, Spiera R, et al:Rituximabversuscyclophosphamidefor ANCA-associated vasculitis.N Engl J Med363:221–232, 2010. doi: 10.1056/NEJMoa0909905 2.Walsh M, Merkel PA, Peh CA, et al: Plasma exchange andglucocorticoidsin severe ANCA-associated vasculitis.N Engl J Med382(7):622-631, 2020. doi:10.1056/NEJMoa1803537 3.Guillevin L, Pagnoux C, Karras A, et al:Rituximabversusazathioprinefor maintenance in ANCA-associated vasculitis.N Engl J Med371:1771–1780, 2014. doi: 10.1056/NEJMoa1404231 