Serum levels of anti-SRP54 antibodies reflect disease activity of necrotizing myopathy in a child treated effectively with combinatorial methylprednisolone pulses and plasma exchanges followed by intravenous cyclophosphamide

Momomura, Mei; Miyamae, Takako; Nozawa, Tomo; Kikuchi, Masako; Kizawa, Toshitaka; Imagawa, Tomoyuki; Drouot, Laurent; Jouen, F.; Boyer, Olivier; Yokota, Shumpei

doi:10.3109/14397595.2013.852852

Cited by 14 publications

(12 citation statements)

References 12 publications

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“…Early detection of anti-SRP antibodies is important for choosing the best treatments. Because anti-SRP antibody titres are correlated with the serum CK levels, the titres could be valuable for monitoring the disease activity (4,10). However, as quantitative tests for anti-SRP antibody titres are still not commonly available, we could not measure the titres.…”

Section: Discussionmentioning

confidence: 97%

Anti-SRP Antibody-positive Myopathy with Universal Alopecia and Multiple Vitiligo

Tokuzumi

Fujisawa²,

Shu³

et al. 2015

Acta Derm Venerol

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Section: Discussionmentioning

confidence: 97%

Anti-SRP Antibody-positive Myopathy with Universal Alopecia and Multiple Vitiligo

Tokuzumi

Fujisawa²,

Shu³

et al. 2015

Acta Derm Venerol

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“…Chronobiological data was collected in half the cases; these occurred between October- February [ 13 , 14 ]. Half reported a preceding infection in the months prior to onset [ 11 – 13 ].…”

Section: Case Presentationsmentioning

confidence: 99%

“…Severe proximal weakness in the absence of typical skin findings of JDM was a universal feature. Common associated symptoms were Raynaud’s (3/8) [ 13 , 15 ], arthritis (3/8) [ 13 , 14 ] and dysphagia (3/8) [ 11 – 13 , 15 ]. 3 children had signs of restrictive lung disease on PFT’s [ 13 – 15 ], although as seen in our cases this may reflect respiratory muscle weakness rather than ILD which is rare in adult anti-SRP syndrome.…”

Section: Case Presentationsmentioning

confidence: 99%

“…Five cases were treated with cyclophosphamide: 1 case developed ovarian failure and treatment was therefore discontinued [ 13 ] and 3 cases were poor responders [ 9 , 13 ]. One patient had a dramatic improvement in muscle strength with combination induction therapy using plasma exchange and methylprednisolone followed by IV cyclophosphamide and azathioprine [ 11 , 12 ]. Azathioprine has also been used successfully as a maintenance agent in combination with IVIG and levflunomide following induction with rituximab [ 15 ].…”

Section: Case Presentationsmentioning

confidence: 99%

“…Long-term outcomes in the existing literature are variable but generally poor. 3/9 patients achieved remission and remained clinically well at the time of publication [ 11 , 12 , 14 , 15 ]. One of these had a period of relapse but subsequently went back into remission [ 15 ].…”

Section: Case Presentationsmentioning

confidence: 99%

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Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature

et al. 2017

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BackgroundAnti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation.Case presentationsThree new patients are reported. All 3 cases presented with profound, rapid-onset, proximal myopathy and markedly raised CK, but no rash. Histology revealed a destructive myopathy characterized by scattered atrophic and necrotic fibres with little or no inflammatory infiltrate. All 3 patients responded to induction with cyclophosphamide, IVIG and rituximab, in conjunction with intensive physiotherapy and methotrexate as the maintenance agent. Our patients regained near-normal strength (MMT > 70/80), in contrast with the current literature where >50% of cases reported severe residual weakness.A literature search on paediatric anti-SRP myositis was performed to June 2016; PubMed was screened using a combination of the following terms: signal recognition particle, autoantibodies, antibodies, myositis, muscular diseases, skeletal muscle, childhood, paediatric, juvenile. Articles in a foreign language were excluded. Nine case studies were found.ConclusionThis paper supports the hypothesis that anti-SRP myositis is distinct from other JIIM. It is an important differential to JDM and should be considered where there is severe weakness without rash or if highly elevated muscle enzymes (CK > 10,000 U/l) are found. Early identification is essential to initiate aggressive medical and physical therapy. Greater international collaboration and long-term follow-up data is needed to establish the most effective treatment strategy for this rare group of patients.

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Therapeutic plasma exchange for the treatment of refractory necrotizing autoimmune myopathy

Kruse

Albayda

Vozniak

et al. 2022

J of Clinical Apheresis

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Introduction Necrotizing autoimmune myopathy (NAM) is strongly associated with pathognomonic autoantibodies targeting 3‐hydroxy‐3‐methylglutaryl‐coenzyme A reductase (HMGCR) or signal recognition particle (SRP), whose levels in turn are correlated with serum creatine kinase (CK) and necrosis. Thus, NAM may be amenable to therapeutic plasma exchange (TPE) to remove pathogenic antibodies and improve patient symptoms. Methods A retrospective case series and literature review of patients presenting with NAM and undergoing treatment with TPE was performed. Clinical data including patient demographics, symptoms, physical exam findings, muscle biopsy, lower extremity imaging, prior therapy, and duration from diagnosis to TPE initiation were collected retrospectively for adult patients with NAM treated with TPE after failing to respond to immunomodulatory therapy. Laboratory data including change in CK levels and myositis‐specific antibody titers from baseline were measured in some patients. Results Six patients (median age at diagnosis 52.5 years, interquartile range [IQR] 35.8‐64.5 years, four male/two female) underwent a median of 7.5 (IQR: 5‐10) TPE procedures with 5% albumin as replacement. All patients exhibited a statistically significant reduction in CK level from pre‐TPE baseline (range: 43.0%‐58.7% reduction). Responses in this cohort were best in patients with antibodies targeting HMGCR and SRP, which are most strongly associated with NAM. These results compare favorably to a literature review of NAM patients (n = 19) treated with TPE, who also exhibited positive clinical and laboratory responses across varying treatment lengths. Conclusion TPE can play a role in the management of NAM, particularly in patients with HMGCR or SRP antibodies who are refractory to pharmacologic immunosuppression.

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Serum levels of anti-SRP54 antibodies reflect disease activity of necrotizing myopathy in a child treated effectively with combinatorial methylprednisolone pulses and plasma exchanges followed by intravenous cyclophosphamide

Cited by 14 publications

References 12 publications

Anti-SRP Antibody-positive Myopathy with Universal Alopecia and Multiple Vitiligo

Anti-SRP Antibody-positive Myopathy with Universal Alopecia and Multiple Vitiligo

Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature

Therapeutic plasma exchange for the treatment of refractory necrotizing autoimmune myopathy

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