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Inflammatory Myopathy
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Overlap myositis is a controversial spectrum of IMs that share two or more variants of myositis (PM, PM/DM, and IMNM). The most common overlapping condition is anti-synthetase syndrome (AS), a distinct special variant of myositis-associated autoantibodies. It consists of a typical collection of clinical symptoms including myositis, Raynaud's disease (RD), arthritis, mechanic's hands (MH), interstitial lung disease (ILD), and presence of anti-tRNA synthetase autoantibodies. The most common of the eight anti-synthetase antibodies is anti-Jo1 antibody. Anti-PL-7 and anti-PL-12 are other rare variants found in PM or the PM/DM spectrum (Table 20.1).
Respiratory
Published in Kristen Davies, Shadaba Ahmed, Core Conditions for Medical and Surgical Finals, 2020
Polymyositis: Inflammatory muscle disorder that is either idiopathic or associated with other connective tissue diseases or malignancy. Features include proximal muscle weakness, Raynaud phenomenon and ILD. Investigations show a ↑ CK and positive anti-Jo1 antibodies. Dermatomyositis is a variant that involves the skin, manifesting in purple papules on the knuckles (Gottron) and a purple heliotrope rash around the eyes.
Nail changes in systemic diseases and drug reactions
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Vascular changes in scleroderma, lupus erythematosus, polyarteritis nodosa,145 and Bürger's disease have to be ruled out. Cholesterol crystal embolization may cause blisters and gangrene of the distal extremities.146,147 Isolated necrotizing vasculitis with neutrophils in the necrotic walls of small superficial vessels may occur.148,149 Small vessel necrotizing vasculitis is seen in Wegener granulomatosis.150,151 The antisynthetase syndrome with anti-Jo1 antibodies may also cause periungual ischemia and fingertip necroses due to severe arteritis.152 Septic vasculitis in gonorrhea and meningitis may involve the nail folds.
Clinical experience in anti-synthetase syndrome: a monocentric retrospective analytical study
Published in Acta Clinica Belgica, 2022
Quentin Maloir, Seidel Laurence, Von Frenckell Christian, Gester Fanny, Louis Renaud, Guiot Julien
The subject characteristics are presented in Table 2. In the whole cohort of 30 patients, we identified 19 with anti-JO1 antibodies, 5 with anti-PL12 antibodies and 6 with anti-PL-7 antibodies. The sex ratio was slightly in favour of males, with all patients being males in the anti-PL7 group (p = 0.021). Interestingly, PL-12 syndrome was significantly more likely to be present in younger patients than those associated with other antibodies (mean age 39,8 vs 53 (JO1) and 73 (PL7) (p = 0.0095)). Overall, 77% of the overall cohort exhibited specific pulmonary involvement. The prevalence of ILD was higher in anti-PL7/PL12-positive patients than in anti-JO1-positive patients but not with a statically significant difference. The former group also exhibited rheumatological signs less frequently. We found no significant difference between ASS patients with anti-JO1 antibodies and those with anti-PL7/PL12 antibodies regarding smoking status, dermatologic signs, malignancy development or blood test results. Anti-JO1-positive patients tended to more commonly exhibit gastroesophageal reflux disease (21% vs. 0%), although the difference was not significant.
Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study
Published in European Clinical Respiratory Journal, 2019
Mads Lynge Jensen, Anders Løkke, Ole Hilberg, Charlotte Hyldgaard, Elisabeth Bendstrup, Dan Tran
Antisynthetase syndrome (AS) is defined by the occurrence of IgG anti-aminoacyl RNA-synthetase (anti-ARS) antibodies and associated types of autoimmune manifestations, mainly myositis. The autoimmunity accounts for important clinical manifestations, such as interstitial lung disease (ILD), arthritis, fever, Raynaud´s phenomenon and mechanic’s hand, which can be present at disease onset or appear later as the disease progresses [1]. At present, eight anti-aminoacyl tRNA-synthetase antibodies (anti-ARS) have been identified: anti-Jo1 (histidyl), anti-PL7 (threonyl), anti-PL12 (alanyl), anti-EJ (glycyl), anti-OJ (isoleucyl), anti-KS (asparaginyl), anti-ZO (phenylalanyl) and anti-YRS/HA (tyrosyl). The most commonly encountered antibody is anti-Jo1 that accounts for up to 60–80% [2–4]. ARS are cytoplasmic enzymes that play a vital role in protein synthesis. Defect of these enzymes can theoretically cause adverse events from every organ. AS is a rare, but probably underdiagnosed disease with a reported prevalence for Caucasians of 87/100,000 in a Norwegian study [5]. The reported incidence was 6–10 per million inhabitants per year diagnosed by presence of anti-ARS and polymyositis/dermatomyositis (PM/DM). Approximately, 25–30% of PM/DM patients had anti-ARS [5]. In a US population, the incidence of AS is considered as low as one in 3–4 million with a diagnostic delay of more than 2 years [6].
Myositis an evolving spectrum of disease
Published in Immunological Medicine, 2018
Simone Barsotti, Ingrid E. Lundberg
The MSAs can be subdivided into groups in their relation to clinical phenotypes. Of the MSAs the autoantibodies that target the tRNA synthetases are the most frequent and called anti-tRNA synthetase autoantibodies, of which anti-histidyl-tRNA synthetase antibodies (anti-Jo1) are the most common, present in up to 20%–30% of patients with IIM. The anti-tRNA synthetase autoantibodies are associated with a distinct clinical phenotype called antisynthetase syndrome (ASS) where myositis is one clinical manifestations and other manifestations are interstitial lung disease (ILD), arthritis, Raynaud´s phenomenon and a skin rash named mechanic’s hands as first described by Dr L. Love [13]. There are now eight identified anti-tRNA synthetase autoantibodies targeting different tRNA synthetases (anti-Jo1, anti-PL7, anti-PL12, anti-EJ, anti-OJ, anti-Ha, anti-Zo and anti-KS; Table 1) [9]. Importantly, an extra-muscular manifestation can be the presenting symptom and can even predominate the clinical phenotype of ASS, and some patients do not experience muscle weakness or develop laboratory signs of a myopathy [14]. In a Spanish cohort 148 of patients with ASS 32.4% presented with ILD, 26.9% with myositis, 17.9% with arthritis and 22.8% with ILD and myositis [7]. Over the years even under immunosuppressive treatment 81.8% had developed ILD and 67.6% had developed ILD and myositis.