To report our experience in using rituximab (RTX) for treating refractory rapidly progressive interstitial lung disease (RP-ILD) complicating anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive amyopathic dermatomyositis (ADM). Medical records of four ADM patients with refractory RP-ILD treated with RTX therapy were reviewed retrospectively. All four patients were tested positive for anti-MDA5 Ab and failed to respond to high-dose systemic steroid and other intensive immunosuppressive therapies. Respiratory symptoms, lung function tests, and high-resolution computed tomography (HRCT) of the chest were compared before and after the first course of RTX. After RTX treatment, all four patients had improvement in the respiratory symptoms in terms of New York Heart Association classification. Two patients successfully had their supplementary oxygen therapy weaned off. The lung function tests were significantly better in all patients. The HRCT showed improvement in three patients while the other one remained static. The recalcitrant vasculitic rashes associated with the anti-MDA5 Ab were also better in all patients. The average daily prednisolone dose dropped from 20 to 6.25 mg post-treatment. None of the patients died throughout the follow-up period which ranged from 6 months to 2 years. However, two patients developed chest infection and one wound infection within 6 months after the RTX infusion. Our results suggest that RTX may be a useful therapy for anti-MDA5 Ab-positive ADM associated with RP-ILD. However, infection is the major risk.
Background The aims of the study were to investigate the prevalence of myositis specific autoantibodies (MSAs) and their associated complications in a cohort of patients with idiopathic inflammatory myopathies (IIMs). Methods A total of 201 consecutive patients with IIMs being followed up in the Rheumatology clinics of the participating regional hospitals in Hong Kong from July 2016 to January 2018 were recruited. Clinical characteristics, treatment history and disease complications were documented. Immunoblot assay was used to detect the MSAs.Results Out of the 201 patients, at least one MSA was found in 63.2% of patients. The most common MSAs were the anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) and the anti-transcriptional intermediary factor 1-gamma antibody (anti-TIF1-γ Ab) (both 13.9%), followed by anti-Jo-1 antibody (12.4%). Anti-MDA5 Ab was present exclusively in dermatomyositis (DM) and was strongly associated with digital ulcers, the clinically amyopathic phenotype and rapidly progressive interstitial lung disease (RP-ILD). Anti-TIF1γ Ab was strongly associated with refractory rash and malignancy. Multivariate analysis showed that the independent risk factors of RP-ILD included anti-MDA5 Ab (OR 14.5, p=0.001), clinically amyopathic DM (OR 13.9, p=0.015) and history of pulmonary tuberculosis (OR 12.2, p=0.026). Cox regression analysis showed that the independent predictors of malignancy included anti-TIF1γ Ab (HR 3.55, p=0.002), DM (HR 3.82, p=0.009) and family history of cancer (HR 3.40, p=0.038). Conclusions MSA testing enables dividing of patients with IIMs into phenotypically homogenous subgroups and prediction of potentially life-threatening complications.
We report a male patient who had refractory idiopathic inflammatory myopathy (IIM) presented with antisynthetase syndrome, being treated by potent immunosuppressants for years, developed Epstein-Barr virus (EBV)-associated lymphoma. Despite the stepping down of the immunosuppressives and active lymphoma therapy, the patient died. On top of the typical association of IIM and malignancy, rare EBV-associated tumors related to EBV infection secondary to the use of potent immunosuppressive therapies could occur. Further investigations are advisable if there are new symptoms and signs or in refractory IIM cases. This report serves as a diagnostic alert that the causation by EBV infection in unusual tumors found in patients with IIM should be considered, as both the treatment and prognosis may differ. A balance between the risks and benefits of immunosuppressive therapies should always be achieved.
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