Clinical Characteristics of Anti-Synthetase Syndrome and Variables Associated with Interstitial Lung Disease and Mortality: A Retrospective Cohort Study

Sodsri, Tulaton; Petnak, Tananchai; Ngamjanyaporn, Pintip

doi:10.3390/jcm12216849

Cited by 7 publications

(5 citation statements)

References 28 publications

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“…Positive results for anti–PL-12, anti–Jo-1, and anti–Ro-52 antibodies were reported in this case. Studies have shown that anti–Ro-52 antibodies in ASS patients are associated with RPILD [ 19 ], whereas anti–PL-12 antibodies are strongly associated with ILD development [ 20 ]. In a previous study, arthritis was the most common initial symptom in the anti–Jo-1 antibody-positive group [ 21 ], and in alignment with literature, our patient presented with multiple joint effusions.…”

Section: Discussionmentioning

confidence: 99%

Anti-synthetase syndrome in a child with pneumomediastinum: a case report and literature review

Lin,

Li,

Fan

et al. 2024

BMC Pulm Med

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Background Anti-synthetase syndrome (ASS) is a group of rare clinical subtypes within inflammatory myopathies, predominantly affecting adult females. Instances of critical illness associated with ASS in children are even rarer. Case presentation We report the case of a 7-year-old boy finally diagnosed with ASS, combined with pneumomediastinum. He presented with intermittent fever persisting for 12 days, paroxysmal cough for 11 days, chest pain, and shortness of breath for 4 days, prompting admission to our hospital. Pre-admission chest CT revealed diffuse pneumomediastinum, subcutaneous pneumatosis in the neck and bilateral chest wall, consolidation, atelectasis, and reticular nodular shadowing in both lungs, as well as pericardial effusion and bilateral pleural effusions. Laboratory tests revealed a positive result for serum MP immunoglobulin M (MP-IgM) and MP immunoglobulin G (MP-IgG). The patient was initially diagnosed with mycoplasma pneumoniae (MP) infection, and following 3 days of antibiotic treatment, the patient's tachypnea worsened. Positive results in muscle enzyme antibody tests included anti–PL-12 antibody IgG, anti–Jo-1 antibody IgG, and anti–RO-52 antibody IgG. Ultrasonography detected moderate effusions in the right shoulder, bilateral elbow, and knee joints. Corticosteroids pulse therapy was initiated on the 27th day following disease onset, and continued for 3 days, followed by sequential therapy for an additional 12 days. The child was discharged on the 43rd day, and subsequent follow-up revealed a significant improvement in consolidation and interstitial lesions in both lungs. Conclusions ASS in children may combine with rapidly progressive interstitial lung disease (RPILD) and pneumomediastinum. It is crucial to promptly identify concurrent immunologic abnormalities during the outbreak of MP, particularly when the disease exhibits rapid progression with ineffective conventional antibiotic therapy.

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Section: Discussionmentioning

confidence: 99%

Anti-synthetase syndrome in a child with pneumomediastinum: a case report and literature review

Lin,

Li,

Fan

et al. 2024

BMC Pulm Med

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“…There are several important points regarding the association between mechanic’s hands and ASS that should be considered: the combination of mechanic’s hands, myopathy, and ILD is not exclusive to ASS; mechanic’s hands can be present in cases without detectable MSAs; the presence of mechanic´s hands, even without satisfying the criteria for ASS, is a risk factor for ILD, a potentially severe and recalcitrant condition if not detected early. [9,25–28,34–36]…”

Section: Discussionmentioning

confidence: 99%

Clinical profiles and treatment outcomes of outpatients with interstitial lung disease and mechanic’s hands: A retrospective and observational cohort

Miranda,

Soares,

Souza

et al. 2024

Medicine

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Idiopathic inflammatory myopathies, especially antisynthetase syndrome, often appear outside of the muscles as interstitial lung disease (ILD). Another typical finding is the presence of mechanic’s hands. The aim of the present study was to describe the clinical, functional, tomographic, and serological data of patients with ILD and mechanic’s hands and their response to treatment and survival rates. This is a retrospective study of ILD with concurrent myopathy. Among the 119 patients initially selected, 51 had mechanic’s hands. All the patients were screened for anti-Jo-1 antibodies. An expanded panel of myopathy autoantibodies was also performed in 27 individuals. Of the 51 patients, 35 had 1 or more antibodies. The most common were anti-Jo-1, anti-PL-7, and anti-PL-12, while of the associated antibodies, anti-Ro52 was present in 70% of the 27 tested individuals. A significant response to treatment was characterized by an increase in predicted forced vital capacity (FVC) of at least 5% in the last evaluation done after 6 to 24 months of treatment. A decrease in predicted FVC of at least 5%, the need for oxygen therapy, or death were all considered treatment failures. All patients were treated with corticosteroids, and 71% with mycophenolate. After 24 months, 18 patients had an increase in FVC, 11 had a decrease, and 22 remained stable. After a median follow-up of 58 months, 48 patients remained alive and three died. Patients with honeycombing on high-resolution chest tomography (log-rank = 34.65; P < .001) and a decrease in FVC ≥5% (log-rank = 18.28, P < .001) had a poorer survival rate. Patients with ILD and mechanic’s hands respond well to immunosuppressive treatment.

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“…Based on a targeted literature review using PubMed, previous studies utilized EIA or LIA alone for detecting anti-Ro52 antibodies and reported the prevalence of anti-Ro52 antibodies as 21.4-65.9% in patients with anti-synthetase antibodies [13][14][15][16][17][18][36][37][38][39][40][41] (Supplementary Table S1) and 31.5-74.7% in those with anti-MDA5 antibodies [13,[19][20][21][22][23][24] (Supplementary Table S2), respectively, which are compatible with the prevalence in the present study.…”

Section: Discussionmentioning

confidence: 99%

“…For the patients with anti-MDA5 antibodies, 33 (97.1%) were either isolated anti-Ro52 positive or anti-Ro52 negative, and only one patient (2.9%) presented with anti-SSA-Ro52 positivity; therefore, we compared clinical characteristics between patients with and without isolated anti-Ro52 positivity (Table 2). Patients with isolated anti-Ro52 positivity were older than those without (65 [61-68] years old vs. 48 [41][42][43][44][45][46][47][48][49][50][51][52][53][54][55][56][57][58][59][60] years old, median [IQR]; p = 0.025). Of note, RP-ILD was more prevalent (75.0% vs. 30.8%, p = 0.042), the partial pressure of oxygen (PaO 2 )/fraction of inspired oxygen (FiO 2 ) ratio was lower (320.0 [270.2-373.4] vs. 416.7 [346.2-437.6], median [IQR]; p = 0.029), and the alveolar-arterial oxygen gradient (A-aDO 2 ) was higher (41.9 [30.4-54.2] vs. 19.2 [10.1-33.6], p = 0.019) in patients with isolated anti-Ro52 positivity than in those without.…”

Section: Clinical Characteristics and Outcomes Stratified By Isolated...mentioning

confidence: 99%

“…Table S2: Previous studies reporting the prevalence and clinical significance of anti-Ro52 antibodies in patients with anti-MDA5 antibodies. References [13][14][15][16][17][18][19][20][21][22][23][24][36][37][38][39][40][41] are cited in the Supplementary Materials. Informed Consent Statement: Informed consent was obtained from all subjects involved in the study.…”

Section: Supplementary Materialsmentioning

confidence: 99%

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Dissociating Autoantibody Responses against Ro52 Antigen in Patients with Anti-Synthetase or Anti-MDA5 Antibodies

Yoshida,

Nagata,

Okazaki

et al. 2023

Diagnostics

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We aimed to dissociate the autoantibody response against the Ro52 protein in patients with anti-synthetase or anti-melanoma differentiation-associated gene 5 (MDA5) antibodies to explore the potential roles of different anti-Ro52 autoantibody responses in disease subclassification. This study used a single-center, prospective myositis cohort involving 122 consecutive patients with anti-synthetase antibodies identified by RNA immunoprecipitation (RNA-IP) and 34 patients with anti-MDA5 antibodies detected using enzyme immunoassay (EIA). Anti-Ro52 antibodies were measured using commercial EIA kits, while anti-Ro/SSA antibodies were identified using RNA-IP. Clinical features and outcomes were stratified according to two different patterns of autoantibody responses against Ro52, including “isolated anti-Ro52”, defined by positive anti-Ro52 and negative anti-Ro/SSA antibodies, and “anti-SSA-Ro52”, defined by positive anti-Ro52 and anti-Ro/SSA antibodies. Isolated anti-Ro52 positivity was the most prevalent autoantibody response in patients with both anti-synthetase (40/122; 32.8%) and anti-MDA5 antibodies (8/34; 23.5%). Isolated anti-Ro52 or anti-SSA-Ro52 positivity was associated with Gottron’s sign in patients with anti-synthetase antibodies, while in patients with anti-MDA5 antibodies, isolated anti-Ro52 positivity was associated with respiratory insufficiency at initial presentation and poor overall survival. Isolated anti-Ro52 positivity could be a potential biomarker for patient stratification; however, the clinical significance of dissociating isolated anti-Ro52 positivity from overall anti-Ro52 positivity was not evident.

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Clinical Characteristics of Anti-Synthetase Syndrome and Variables Associated with Interstitial Lung Disease and Mortality: A Retrospective Cohort Study

Cited by 7 publications

References 28 publications

Anti-synthetase syndrome in a child with pneumomediastinum: a case report and literature review

Anti-synthetase syndrome in a child with pneumomediastinum: a case report and literature review

Clinical profiles and treatment outcomes of outpatients with interstitial lung disease and mechanic’s hands: A retrospective and observational cohort

Dissociating Autoantibody Responses against Ro52 Antigen in Patients with Anti-Synthetase or Anti-MDA5 Antibodies

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