О р и г и н а л ь н ы е и с с л е д о в а н и я ФГБНУ Научноисследовательский институт ревматологии им. В.А. Насоновой,
BackgroundInterstitial lung disease (ILD) is the most common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course of the disease. IIM patients with ILD often show resistance to conventional treatment with glucocorticoids (GC) and cytotoxic drugs so addition of biologic agents is an interesting possibility.ObjectivesTo assess efficacy and tolerability of rituximab (RTM) in IIM patients with ILD.MethodsIIM patients fulfilling Bohan and Peter criteria and having ILD were followed up in the Nasonova Research Institute of Rheumatology from 2009 to 2015. RTM was administered in case of intolerance or inadequate response to GC and other immunosuppressive drugs. Manual muscle testing (MMT), dyspnea assessment according to NYHA, creatinkinase (CK) and anti-Jo-1 antibodies (anti-Jo-1) assay; forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) evaluation as well as high-resolution computed tomography (HRCT) scanning of the chest were performed at baseline, 6–12 and 18–24 months after inclusion.Results41 patients (34 females, 7 males) with median age 50 [44;56], median disease duration 24 months [7;108] were included. 28 from them had decreased MMT value and class 1–2 NYHA dyspnea was present in 19. 18 patients were positive for anti-Jo-1 and 15 had elevated CK level. HRCT showed ILD signs including ground glass opacities (GGO) in all cases. FVC<80% was revealed in 16 and DLCO<80% - in 32 cases. All patients received GC 10–90 mg/day equivalent to prednisolone, 28 were treated with immunosuppressive agents (cyclophosphamide or mycophenolate mofetil). Patients received RTM courses consisted from 2 infusions of RTM 1000 or 500 mg at days 0 and 14. 1 RTM course was provided in 13, 2 – in 10, 3 – in 11, 4 – in 2, 5 – in 2, and 8 – in 3 patients. The follow up lasted for at least 12 months in 33, and for at least 24 months – in 15 patients. Dyspnea subsided and CK level decreased to normal values in all patients within 6–12 months after the first RTM infusion. 16 patients improved FVC by >10%, and 7 demonstrated DLCO increase by >10%. Worsening of pulmonary function tests was documented in 2 cases, while in the rest they were stable. After 18–24 months of follow up FVC remained +10% better vs baseline value in 10, DLCO – in 8 patients. In the rest patients these parameters remained stable. ILD was not progressing in 27 patients based on HRCT images in 6–12 months, moreover GGO lesions count was reduced in 10 patients, but there were cases of ILD worsening documented in 6 patients. Although, HRCT monitoring in 18–24 months showed ILD improvement in 7 patients and worsening – in 2, in remaining patients ILD was stable. Comorbid infections were documented in 18 patients, commonly manifesting in 2–3 months after the first RTM infusion.ConclusionsRTM treatment allows to achieve stable disease without typical for this condition further progression of pulmonary lesions in the majority of IIM patients with ILD.Disclosure of InterestNone declared
Идиопатические воспалительные миопа-тии (ИВМ) -группа редких аутоиммунных ге-терогенных заболеваний неизвестной этиоло-гии, характеризующихся воспалительным по-ражением поперечно-полосатой мускулатуры и кожи, а также специфической органной па-тологией [1,2].Клиническая картина ИВМ характеризу-ется прогрессирующей симметричной мы-шечной слабостью проксимальных отделов конечностей (что ведет к обездвиженности пациентов), мышц шеи, дисфагией, измене-нием голоса (назолалией), параорбитальным отеком, формированием сгибательных сухо-жильно-мышечных контрактур.Помимо перечисленных клинических проявлений, общих для ИВМ, наблюдается фенотипическая неоднородность их подти-пов, связанная с различными морфологи-ческими и серологическими характеристи-ками [3], определяющими особенности ма-нифестации, течения и прогноза [4,5]. Фармакотерапия базируется на примене-нии высоких доз глюкокортикоидов (ГК; ≥1 мг/кг/сут) в сочетании с иммуносупрес-сивными препаратами. Терапевтическая та- 191П р о г р а м м а н е п р е р ы в н о г о п о с л е д и п л о м н о г о о б р а з о в а н и я в р а ч е й В лекции рассматривается проблема редких системных заболеваний соединительной ткани -идиопатиче-ских воспалительных миопатий (ИВМ). Подчеркивается клинико-иммунологическая неоднородность их подтипов, определяющая терапевтическую тактику и прогноз. Представлены диагностические критерии ИВМ. Предлагается алгоритм дифференциальной диагностики, который базируется на исключении феноти-пически сходных форм миопатий различного генеза. Ключевые слова: идиопатические воспалительные миопатии; полимиозит; дерматомиозит; антисинтетазный синдром; некротизирующая миопатия; миозит с включениями; дифференциальная диагностика миопатий. Для ссылки: Антелава ОА. Полимиозит/дерматомиозит: дифференциальная диагностика. Научно-практиче-ская ревматология. 2016;54(2):191-198. POLYMYOSITIS/DERMATOMIOSITIS: DIFFERENTIAL DIAGNOSISAntelava O.A.The lecture considers the problem of rare systemic connective tissue diseases, such as idiopathic inflammatory myopathies (IIMs). It underlines the clinical and immunological heterogeneity of their subtypes, which defines therapeutic tactics and prognosis. The diagnostic criteria for IIMs are given. A differential diagnostic algorithm based on the exclusion of phenotypically similar forms of myopathies of different genesis is proposed.
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