Rituximab therapy in patients with refractory dermatomyositis or polymyositis: differential effects in a real-life population

Unger, L.; Kampf, S.; Lüthke, K.; Aringer, Martin

doi:10.1093/rheumatology/keu024

Cited by 74 publications

(47 citation statements)

References 22 publications

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“…Moreover, a subsequent subgroup analysis has highlighted that response to rituximab may be influenced by serological phenotype with ASS and antiMi2 patients more likely to gain benefit [122]. A therapeutic role for rituximab within the different clinicoserological phenotypes has been further emphasised in a number of series, including refractory patients with ASS-ILD, anti-MDA5 and anti-SRP disease [117,[123][124][125][126][127][128][129]. Therefore, it seems that stratification based on autoAbs can act as predictive markers of clinical response to treatment, which again highlights their diagnostic utility.…”

Section: Does Clinico-serological Phenotype Influence Outcome and Trementioning

confidence: 99%

The Clinical Features of Myositis-Associated Autoantibodies: a Review

Gunawardena

2015

Clinic Rev Allerg Immunol

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The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

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Section: Does Clinico-serological Phenotype Influence Outcome and Trementioning

confidence: 99%

The Clinical Features of Myositis-Associated Autoantibodies: a Review

Gunawardena

2015

Clinic Rev Allerg Immunol

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“…Concerning ILD associated with CTDs, amelioration of both PFT and HRCT score extent was reported after rituximab administration in a monocentric cohort of 34 patients affected by anti-synthetase syndrome; the best outcome was observed in case of disease duration less than 12 months and/or in case of acute onset/ exacerbation of ILD [8]. The beneficial effect of rituximab in ILD secondary to PM/DM was reported in other case series [9]. The mortality from infection remains a big concern [8,9].…”

Section: Discussionmentioning

confidence: 62%

“…The beneficial effect of rituximab in ILD secondary to PM/DM was reported in other case series [9]. The mortality from infection remains a big concern [8,9]. In SSc, rituximab treatment improved skin involvement and ameliorated or prevented the further decline of pulmonary function in case of lung disease [10][11][12][13].…”

Section: Discussionmentioning

confidence: 81%

Dramatic improvement of anti-SS-A/Ro-associated interstitial lung disease after immunosuppressive treatment

et al. 2016

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The aim of the study was to report three patients affected by interstitial lung disease associated with positive anti-SS-A/Ro autoantibody who showed a dramatic improvement after immunosuppressive treatment. Medical charts were reviewed to obtain clinical data, laboratory parameters, lung function tests, high-resolution computed tomography results and response to immunosuppressive treatment. The three patients showed a clinical picture of a lung-dominant connective tissue disease characterized by a sudden onset with dyspnea, cough and subtle extrathoracic features together with positive anti-SS-A/Ro antibody and weak titer antinuclear antibodies. All three patients responded favorably to immunosuppressive therapy: Two cases were treated with a combination of corticosteroid and cyclophosphamide followed by mycophenolate mofetil; in the third patient, clinical benefit was obtained after rituximab was added to corticosteroid and immunosuppressant drug. In spite of an abrupt onset with significant lung function impairment, all three patients had a favorable clinical response to immunosuppressive therapy. This report may be useful in making therapeutic decisions in case of interstitial lung disease associated with anti-SS-A antibody.

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“…Even though RTX has been successfully used in the treatment of cutaneous ulcerations in diseases, such as refractory cases with LEP, polyarteritis nodosa, polymyositis/dermatomyositis and ANCA-associated vasculitis, the etiological association of rituximab with these ulcers is still unclear and controversial. [20][21][22] Therefore, we suggest that these atypical ulcers could be a rare mysterious clinical sign of SLE. We think that the cutaneous ulcers reported in the patient in the current paper may also have occurred as a result of necrobiotic changes in the subcutaneous tissue caused by vascular changes.…”

Section: Discussionmentioning

confidence: 93%