What should we expect when two myositis-specific antibodies coexist in a patient.

Eur J Med Res

Neurology Department, Peking University First Hospital, No. 8 Xishiku Street, Xicheng District, Beijing, 100034, China.

Published: October 2023

Background: The coexistence of two myositis-specific autoantibodies (MSA) is considered extremely rare. We describe three patients with both anti-signal recognition particle (SRP) antibodies and another MSA in serum.

Methods: We performed a retrospective clinical data collection and follow-up studies of the clinical manifestations and treatment outcome of three patients positive with anti-SRP antibodies and other MSAs. IgG antibodies against MSAs were detected using commercial line immunoblot assay.

Results: The tests of MSA showed positive result of anti-SRP antibodies and another one MSA including anti-TIF1-γ, anti-Jo1, or anti-EJ antibodies, respectively. The proximal muscle weakness appeared in 2 patients; interstitial lung disease presented in 2 patients. The serum CK level was elevated in 1 patient. The muscle biopsy showed necrotizing myopathy in 1 patient and deposition of membrane attack complex on scattered myofibers in the other one patient. One of the two patients with interstitial lung disease died because of respiratory failure. One patient had completely improved and the other one showed partial remission after immunosuppressive therapy.

Conclusions: The patients with anti-SRP antibodies co-occurred with the other MSA may have various clinical characteristics. The clinicopathological phenotypes of these patients seem to be mainly caused by one of the MSAs, namely the responsible antibody.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10571367PMC
http://dx.doi.org/10.1186/s40001-023-01363-5DOI Listing

Publication Analysis

Top Keywords

anti-srp antibodies
12
three patients
8
antibodies msa
8
antibodies msas
8
patients interstitial
8
interstitial lung
8
lung disease
8
antibodies
7
patients
7
patient
5

Similar Publications

Myositis-specific autoantibodies play an important role on the disease phenotype of idiopathic inflammatory myopathies (IIMs). Anti-signal recognition particle (SRP) antibody-positive patients with IIMs may present with severe myopathy and highly elevated serum creatine kinase levels. These patients are often resistant to immunosuppressive therapy, but there is no established treatment strategy.

View Article and Find Full Text PDF
Article Synopsis
  • - Overlap syndrome of systemic sclerosis and idiopathic inflammatory myopathies is becoming more common, but its link with immune-mediated necrotizing myositis is not well-documented, requiring careful differentiation for proper treatment and prognosis.
  • - Anti-signal recognition particle (anti-SRP) antibodies are crucial in diagnosing immune-mediated necrotizing myopathy, causing muscle damage and weakness, while their relationship with cardiac issues remains inconclusive, complicating potential management.
  • - Diagnosing heart complications like myocarditis in these patients is challenging, often relying on cardiac imaging rather than biopsies, and this paper explores the issues of accurately diagnosing and treating diseases related to collagen and anti-SRP antibodies.
View Article and Find Full Text PDF

Objective: Idiopathic inflammatory myopathies (IIMs) are a group of heterogeneous, systemic autoimmune diseases characterized by specific clinical features and, frequently, skeletal muscle inflammation. Specific subtypes of IIMs can be characterized by myositis-specific autoantibodies and are associated with distinct clinical phenotypes. Here, we focus on anti-melanoma differentiation-associated protein 5 (MDA5)-positive myositis and anti-signal recognition particle (SRP)-positive myositis, both of which exhibit seasonality but lack known environmental triggers.

View Article and Find Full Text PDF

Objective: We aimed to explore the efficacy and safety of efgartigimod in patients with refractory immune-mediated necrotizing myopathy (IMNM).

Methods: This open-label pilot observational study included seven patients with refractory IMNM, all of whom received intravenous efgartigimod treatment. The clinical response was assessed after 4 weeks of efgartigimod treatment according to the 2016 American College of Rheumatology-European League Against Rheumatism response criteria for adult idiopathic inflammatory myopathy.

View Article and Find Full Text PDF

Immune-mediated necrotizing myopathy (IMNM), an inflammatory muscle disease, typically presents as severe muscle weakness due to immunologic mechanisms. Some cases also show cutaneous manifestations, interstitial lung disease, and sicca symptoms. In this report, we present a unique case of an elderly man with a history of mild and stable Sjögren's syndrome (SS) for over 10 years, who later developed interstitial lung disease and myositis, leading to a diagnosis of IMNM confirmed by the presence of anti-signal recognition particle antibodies.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!