Camptocormia with trigeminal involvement revealing myositis with anti-Ku antibodies

Chaves, S. De Almeida; Moulis, Guillaume; Pugnet, G.; Sailler, L.; Astudillo, L.

doi:10.1016/j.jbspin.2018.03.013

Cited by 3 publications

(1 citation statement)

References 5 publications

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“…Common clinical features of anti-Ku positive patients with IIMs included myalgia (91%), proximal muscle weakness (89%), and dysphagia (36%). Rare symptoms such as camptocormia were also found in anti-Ku positive patients (39,40). The anti-Ku associated overlap syndrome includes a wide range of CTDs including scleroderma, Sjögren syndrome and systemic lupus erythematosus (SLE) (41).…”

Section: Maas In Overlap Myositismentioning

confidence: 99%

Narrative review of autoantibodies in idiopathic inflammatory myopathies

Liu¹,

Zheng²,

Hao³

et al. 2023

Ann Transl Med

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Objective: To discuss the characteristics of autoantigens, detection methods and roles of myositis associated autoantibodies (MAAs) and myositis specific autoantibodies (MSAs), as well as the clinical features of disease subgroups defined by MAAs/MSAs. Background: Autoantibodies in patients with idiopathic inflammatory myopathies (IIMs) are conventionally divided into MAAs and MSAs. MAAs usually refer to autoantibodies which are also available in systematic autoimmune diseases (anti-PM/SCL, anti-Ku, anti-Ro52 and anti-U1RNP antibodies). MSAs refer to autoantibodies which were distinctive for IIM (anti-Mi-2, anti-MDA5, anti-TIF1gammma, anti-NXP2, anti-SAE, anti-synthetase, anti-SRP, anti-HMGCR and anti-cN1A antibodies). The discovery and identification of novel autoantigens is a long and complicated process, which brought light in immunopathogenesis of IIMs. Detection methods of MAAs/MSAs mainly consist of monospecific methods [immunoprecipitation, enzyme linked immune sorbent assay (ELISA) and indirect immunofluorescence] and multispecific methods [line immunoassay (LIA), dot immunoassay (DIA) and addressable laser bead assay (ALBIA)]. Patients with different MAAs/MSAs have different clinical features and require different clinical management.Methods: The search engine PubMed (https://www.ncbi.nlm.nih.gov/pmc/) was used to research the keywords "autoantibodies", "idiopathic inflammatory myopathies", "detection methods" and "clinical features". A narrative review was conducted to literature findings from 1975 to 2020. Conclusions: Development and validation of efficient detection methods of MAAs and MSAs help clinicians for diagnosis, classification and management of IIMs. The exploration of clinical features associated with different autoantibodies that facilitate the creation of diagnostic and classification guidelines and further clinical decision-making is of high value.

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Section: Maas In Overlap Myositismentioning

confidence: 99%

Narrative review of autoantibodies in idiopathic inflammatory myopathies

Liu¹,

Zheng²,

Hao³

et al. 2023

Ann Transl Med

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Anti-Ku antibody-positive myositis presenting as a wide range of axial myopathies and myocarditis: A case report and review of the literature

Kono

Komai

Yuki

et al. 2021

Modern Rheumatology Case Reports

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Idiopathic inflammatory myopathies (IIMs) are autoimmune diseases predominantly affecting proximal muscles; paraspinal muscle involvement is relatively rare. Because paraspinal myopathies do not always cause clinically-evident symptoms, the diagnosis of IIMs with axial myopathies can be challenging. Anti-Ku autoantibodies, initially reported in polymyositis/systemic sclerosis overlap syndrome, are myositis-associated antibodies (MAAs) observed in patients with a wide variety of connective tissue diseases (CTDs). Few reports have been published demonstrating predominant axial myopathy in IIM patients with anti-Ku antibodies. Herein, we investigated a previously healthy Japanese woman in her early 70s who presented with Raynaud’s phenomenon, back pain, and exertional dyspnea. The creatine kinase (CK) was elevated and antinuclear antibody staining was positive, but myositis-specific antibodies (MSAs) were negative. Magnetic resonance imaging (MRI) revealed myocarditis and a wide range of axial muscle inflammation, including bilateral thoracolumbar paraspinal, infraspinatus, and trapezius muscles. The muscle biopsy was consistent with IIM. In addition, anti-Ku antibody was positive. Administration of prednisolone and tacrolimus quickly alleviated the symptoms and the CK level returned to normal. The diagnosis of IIM was arduous in this case because she did not present with camptocormia, muscle weakness involving the proximal limbs was not apparent, and MSAs were negative. Whether axial myopathy and myocarditis are more prevalent in IIM patients with than without anti-Ku antibodies is uncertain. Clinicians should suspect axial myopathy and MAAs, such as anti-Ku antibodies, especially in patients in whom muscle weakness of the proximal limbs is not noticeable.

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Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study

et al. 2023

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Objectives Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management. Methods This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC). Results Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5–75.0); 21 were female (77.8%). There were 9 inclusion-body myositis (IBM, 33.3%), 9 overlap myositis (OM, 33.3%), 5 dermatomyositis (DM, 18.5%), 2 immune checkpoint inhibitor-related myositis (7.4%), 1 focal myositis (3.7%) and 1 myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤ 70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patient (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged > 70 years at first symptoms, there were 8 IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC. Conclusion The analysis of IM patients with inaugural DHS/CC delineates 2 groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications.

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Camptocormia with trigeminal involvement revealing myositis with anti-Ku antibodies

Cited by 3 publications

References 5 publications

Narrative review of autoantibodies in idiopathic inflammatory myopathies

Narrative review of autoantibodies in idiopathic inflammatory myopathies

Anti-Ku antibody-positive myositis presenting as a wide range of axial myopathies and myocarditis: A case report and review of the literature

Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study

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