ABSTRACT
Background. Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. Objectives. To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. Methods. Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8+/-11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) 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, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jo-1 antibody. All pts received prednisolone at a mean dose of 24.3+/-13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18 , mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. Results. The mean follow-up period after the first infusion of RTX was 47.2+/-11.9 months. Pts received 1-11 courses of RTX . The cumulative mean dose of RTX was 4.6 +/-2.5g. MMT 8 increased from 135.8+/-13.5 to 148.75+/-3.5 (p=0.000001). CK level decreased DELTACK - 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4+/-37 to 96.5+/-79 u/ml (p=0.00002), FVC increased from 82+/-22.6 to 96,9+/-22% (p=0.00011). DLCO increased from 51.4+/-15.2 to 60+/-77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3+/-13 to 5.7+/-2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. Conclusions. The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible.
ABSTRACT
SESSION TITLE: Treatment Debates in Critical Care SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/17/2022 12:15 pm - 1:15 pm PURPOSE: Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. OBJECTIVES:To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. METHODS: Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8±11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) 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, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jо-1 antibody. All pts received prednisolone at a mean dose of 24.3±13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18, mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. RESULTS: The mean follow-up period after the first infusion of RTX was 47.2±11.9 months. Pts received 1-11 courses of RTX. The cumulative mean dose of RTX was 4.6 ±2.5g. MMT 8 increased from 135.8±13.5 to 148.75±3.5 (p=0.000001). CK level decreased ΔCK – 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4±37 to 96.5±79 u/ml (p=0.00002), FVC increased from 82±22.6 to 96,9±22% (p=0.00011). DLCO increased from 51.4±15.2 to 60±77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3±13 to 5.7±2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. CONCLUSIONS: The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. CLINICAL IMPLICATIONS: RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible DISCLOSURES: No relevant relationships by Lidia Ananyeva No relevant relationships by Maria Aristova No relevant relationships by Oxana Desinova No relevant relationships by Liudmila Garzanova No relevant relationships by Anna Khelkovskaya-Sergeeva No relevant relationships by Olga Koneva No relevant relationships by Dmitry Kulikovsky
ABSTRACT
SESSION TITLE: Systemic Disease with Diffuse Lung Symptoms Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Rapidly progressive interstitial lung disease (RP-ILD) is a rare and potentially fatal manifestation of dermatomyositis (DM) and has considerable impact in terms of the prognosis. CASE PRESENTATION: A 52-year-old male demonstrated DM-typical rash, fever, mialgias, and mild muscle weakness 3 months after asymptomatic COVID-19 infection. Two weeks later dysphonia and progressive dyspnea appeared. Lung CT scan showed the picture of organizing pneumonia. His COVID-19 PCR test was negative multiple times. Laboratory tests revealed the following numbers: ALT 210 IU/L, AST 748 IU/L, LDH 613 IU/L, CPK 1165 IU/L, ferritin 1145ϻg/l, CRB 11 mg/l. The patient was tested positive for anti-Ro52 antibodies, while anti-synthetase and scleroderma-associated antibodies were not discovered. Anti-melanoma differentiation-associated gene 5 (MDA5) test was not available due to the lack of the necessary test systems in the country. The patient was diagnosed with DM. Combined immunosuppressive therapy was administered, including: oral prednisolone 60 mg per day and 720 mg intravenously, dexamethasone 64-24 mg intravenously per diem, ciclosporin 200 mg и cyclophosphamide 600 mg, and 3 plasmapheresis sessions followed by an intravenous immunoglobulin. As a result of the therapy, muscle weakness disappeared and CPK levels returned to normal limits, however dyspnea progressed and ferritin levels hit 3500ϻg/l. After the following 3 weeks of intensive combined immunosuppressive therapy, the patient demonstrated symptoms of severe respiratory failure (RF). CT scan showed multiple traction bronchiectasis, wide areas of ground glass opacity, pneumomediastinum and subcutaneous emphysema of a neck and supraclavicular regions. Ciclosporin was replaced with tofacitinib with the dose of 10 mg per diem, IL-6 inhibitor (olokizumab 256 mg) was injected intravenously, massive broad-spectrum antibiotic therapy was administered. RF progressed and the patient was put on mechanical ventilation. The patient died of acute RF and sepsis a week later. DISCUSSION: RP-ILD is a common manifestation of severe MDA5+ DM, which is also associated with necrotizing vasculitis and amyopathic/hypomyopathic muscle involvement. In this case acute ILD in a patient with typical DM could also have been provoked by previous COVID-19 infection. CONCLUSIONS: The courses of disease for COVID-19 and MDA5+ DM have several similarities, which means it can be the same for their pathogenesis and clinical manifestations. In spite of early screening and intensive immunosuppressive therapy in such cases, the prognosis of patients with DM and RP-ILD is still poor and is associated with high mortality. Reference #1: Wang G, Wang Q, Wang Y, et al. Presence of Anti-MDA5 Antibody and Its Value for the Clinical Assessment in Patients With COVID-19: A Retrospective Cohort Study. Front Immunol. 2021 Dec 20;12:791348. doi: 10.3389/fimmu.2021.791348. PMID: 34987516;PMCID: PMC8720853. DISCLOSURES: No relevant relationships by Lidia Ananyeva No relevant relationships by Maria Aristova No relevant relationships by Liudmila Garzanova No relevant relationships by Anna Khelkovskaya-Sergeeva No relevant relationships by Dmitry Kulikovsky