Plasma-exchange as a “rescue therapy” for dermato/polymyositis in acute phase. Experience in three young patients

Cozzi, Franco; Marson, Piero; Pigatto, Erika; Tison, Tiziana; Polito, Pamela; Galozzi, Paola; Silvestro, Giustina De; Punzi, Leonardo

doi:10.1016/j.transci.2015.07.005

Cited by 12 publications

(5 citation statements)

References 17 publications

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“…Our results indicated that 63.2% (24/38) patients achieved PR, which is an inspiring result due to the high fatality rate of severe DM/PM. The positive effects of TPE in acute phase of DM/PM were confirmed by several anecdotal cases, [15][16][17]32 although Miller et al 21 reported that TPE failed to improve functional capacity or muscle strength in one RCT consisting of 39 subjects. It is uncertain whether TPE is responsible for remission in idiopathic inflammatory myositis, even with those favorable outcomes.…”

Section: Discussionmentioning

confidence: 91%

“…In some cases, TPE showed important advantages, when patients presented with inflammatory storm or certain lifethreatening acute complications, such as rapidly progressive interstitial lung disease (RP-ILD) or diffuse alveolar hemorrhage (DAH). [15][16][17][18] However, due to insufficient large-scale trials on TPE, there is no specific recommendations associated with apheresis for most rheumatic disorders. Besides, there is, thus far, no uniform database recording the TPE-associated adverse events (AEs) and benefits of TPE in the treatment of rheumatic disorders.…”

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confidence: 99%

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Experience of therapeutic plasma exchange in rheumatic diseases: Albumin may be a suitable substitute for plasma

2021

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Objectives: In this study, we aimed to assess the value of therapeutic plasma exchange (TPE) in the treatment of rheumatic diseases and compare the safety of different replacement fluids used in TPE. Patients and methods: A total of 727 TPE procedures in 285 patients (57 males, 228 females; mean age: 39.7±15.4 years; range, 13 to 79 years) with rheumatic diseases between January 2011 and February 2019 were retrospectively analyzed. Data including demographic and clinical characteristics of the patients were recorded. Treatment response to TPE and adverse events were evaluated in all patients. Results: Indications for TPE included 13 different disorders, with the majority being systemic lupus erythematosus (up to 50%). The mean number of TPE sessions was 2.55±1.00 per patient and the mean exchange plasma volume was 2,270±256 mL per session. Combined plasma and albumin was the most frequently used replacement fluid (69.5%), followed by albumin and plasma in 20.5% and 10.0% of episodes, respectively. Up to 73.7% (210/285) patients achieved clinical improvement after TPE treatment. Adverse events occurred in 15.1% (110/727) of all the procedures, and allergic reaction (34.5%) was the most common event. The overall incidence rate of complication was similar among the three types of replacement fluids (p=0.214). Conclusion: Based on our study results, TPE is an invasive, but safe, useful and, sometimes, essential tool with an acceptable risk/benefit ratio for most rheumatic diseases. Albumin can be used as a feasible substitute for plasma in case of shortage of blood resources.

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Section: Discussionmentioning

confidence: 91%

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confidence: 99%

Experience of therapeutic plasma exchange in rheumatic diseases: Albumin may be a suitable substitute for plasma

2021

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“…Cherin investigated 27 patients who suffered from severe pharyngeal muscle weakness and were resistant to conventional therapy; eight (30%) reported the disappearance of symptoms, whereas the other 19 (70%) reported the stabilization of dysphagia after receiving TPE (15). Some case reports have also showed that TPE in association with immunosuppressant agents could play a relevant role in severe pharyngo-esophageal muscle weakness (28).…”

Section: Discussionmentioning

confidence: 99%

Efficiency of Therapeutic Plasma-Exchange in Acute Interstitial Lung Disease, Associated With Polymyositis/Dermatomyositis Resistant to Glucocorticoids and Immunosuppressive Drugs: A Retrospective Study

et al. 2019

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Interstitial lung disease (ILD) is a life-threating complication, commonly associated with polymyositis (PM), and dermatomyositis (DM). A subset of acute ILD associated with PM/DM patients are refractory to conventional treatment, and leads to a high rate of mortality. The efficacy of therapeutic plasma-exchange (TPE) as a PM/DM treatment to improve muscle involvement is controversial due to a lack of evidence. However, in recent reports, TPE has been effective in improving lung involvement. To evaluate the efficacy of this therapy, we retrospectively studied TPE treatment outcomes for in 18 acute PM/DM-ILD patients who were resistant to conventional therapies. Five patients were diagnosed with DM (27.8%), 11 with CADM (61.1%), and two with PM (11.1%). Among 18 patients, 11 (61.1%) achieved satisfactory improvement after four or more rounds of TPE, whereas seven died due to respiratory failure. We also analyzed risk factors to predict unresponsiveness to TPE in these patients. Notably, the prevalence of subcutaneous/mediastinal emphysema was significantly higher in the non-responsive group (6/7, 85.7%) than in the responsive group (2/11, 18.2%; P = 0.013); moreover, patients with this complication were mainly in the CADM subgroup (6/8, 75%). Subcutaneous/mediastinal emphysema and increased serum ferritin levels were shown to be poor prognostic factors, predictive of unresponsiveness to TPE, in PM/DM patients. No autoantibodies were found to be associated with TPE outcome, although we only investigated anti-Jo-1 and anti-Ro antibodies; the clinical significance of other myositis-specific autoantibodies, especially anti-melanoma differentiation-associated gene 5 (MDA5) antibody, is not known. Our results indicate that TPE might be an alternative treatment for acute PM/DM-ILD patients resistant to conventional therapies, except for those with subcutaneous/mediastinal emphysema and high serum ferritin levels.

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“…68 A current paper from 2015 reported three cases of myositis with severe pharyngo-esophageal muscle weakness, where plasma exchange was used successfully as a rescue therapy. 69 Plasma exchange is not a common therapy in myositis. But it may serve as a possible treatment in acute forms of myositis where the conventional therapy has failed or cannot be applied due to side effects.…”

Section: Plasma Exchange In Myositismentioning

confidence: 99%

“…A current paper from 2015 reported three cases of myositis with severe pharyngo-esophageal muscle weakness, where plasma exchange was used successfully as a rescue therapy. 69…”

Section: Treatment Of Dm Pm and Om Including Assmentioning

confidence: 99%

New insights into the treatment of myositis

Glaubitz

Zeng

Schmidt

2020

Therapeutic Advances in Musculoskeletal

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The myositis syndromes include polymyositis, dermatomyositis (DM), necrotizing myopathy, inclusion body myositis (IBM), antisynthetase syndrome and overlap syndromes with myositis. These syndromes mostly occur in middle-aged patients, while juvenile DM occurs in children and adolescents. Patients mostly show a subacute weakness and myalgia in the upper and lower limbs, the diagnosis is based upon these clinical findings in combination with muscle biopsy results and specific serum autoantibodies. In recent years, research achieved a better understanding about the molecular mechanism underlying the myositis syndromes, as well as disease progress and extramuscular organ manifestations, such as interstitial lung disease and association with neoplasias. Treatment mainly consists of glucocorticosteroids and immunosuppressants. IBM is usually refractory to treatments. This review provides an overview of the current standards of treatment and new treatment options like monoclonal antibodies and new molecular therapies and their first results from clinical trials.

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Plasma-exchange as a “rescue therapy” for dermato/polymyositis in acute phase. Experience in three young patients

Cited by 12 publications

References 17 publications

Experience of therapeutic plasma exchange in rheumatic diseases: Albumin may be a suitable substitute for plasma

Experience of therapeutic plasma exchange in rheumatic diseases: Albumin may be a suitable substitute for plasma

Efficiency of Therapeutic Plasma-Exchange in Acute Interstitial Lung Disease, Associated With Polymyositis/Dermatomyositis Resistant to Glucocorticoids and Immunosuppressive Drugs: A Retrospective Study

New insights into the treatment of myositis

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