Anti-Synthetase Syndrome Complicated by Pyogenic Myositis

2021 ◽  
Vol 18 ◽  
Author(s):  
David Lizarazo ◽  
Karen Cifuentes ◽  
Paula Andrea Forero ◽  
Hernan Páez
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy ◽  
Autoimmune Disorder ◽  
Joint Involvement ◽  
Fatty Tissue ◽  
Trna Synthetases ◽  
Tissue Replacement ◽  
Antisynthetase Antibodies ◽  
Optimal Approach

Background: Anti-synthetase syndrome is a rare autoimmune disorder characterized by autoantibodies against aminoacyl-tRNA-synthetases. Inflammatory myopathy and interstitial lung disease could be present among other manifestations. Anti-Jo-1 is the most common antisynthetase antibody and is the most likely to present with the classic triad (interstitial lung disease, myositis, and arthritis) and have more muscle and joint involvement than patients with other antisynthetase antibodies. Case report: Here, we present a case of a 60-year-old female patient, with a previous diagnosis of myositis, secondary to the anti-synthetase syndrome, with a complication by pyogenic myositis. Conclusion: Diagnosis is made by a multidisciplinary approach, occasionally muscle and/or lung biopsy are needed. Imaging studies, Especially magnetic resonance imaging, based on findings such as muscle and fascial edema, and fatty tissue replacement, allow an optimal approach.

scholarly journals 14. Digital ischemia and interstitial lung disease in antisynthetase syndrome with PL-12 antibody

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Maria Usman Khan ◽  
David McCormick
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features ◽  
Conflicts Of Interest ◽  
Inflammatory Myopathy ◽  
Antisynthetase Syndrome ◽  
Raynaud Phenomenon ◽  
Trna Synthetases ◽  
Aminoacyl Trna Synthetases ◽  
Digital Ischemia

Abstract Introduction Antisynthetase syndrome (ASS) is a rare idiopathic inflammatory myopathy with nearly 89% showing interstitial lung disease (ILD). The hallmark of ASS is the presence of serum autoantibodies directed against aminoacyl-tRNA synthetases that include Jo-1 (most commonly detected), PL-7, PL-12, OJ, EJ, KS, Wa, YRS and Zo. However, in a small subpopulation without evidence of myositis, the diagnosis may be critically delayed, hindering management of this rapidly progressive disease. We report an interesting case of anti-PL-12/anti-SSA 52kD ASS presenting as acute digital ischemia, an association rarely described previously. In cases with ILD, the severity of lung condition generally determines the prognosis. Case description A 77-year-old Caucasian female presented with sudden onset of painful, blue discolouration in her bilateral fingertips two-weeks after mild lower respiratory tract infection and occasional pyrexia. She was a non-smoker, otherwise independent lady who had background history of ischemic heart disease, diverticulosis and hypertension. Her physical examination revealed dusky blue digits and dry ulceration. She had extensive investigations that showed raised CRP (61mg/L), eGFR 39ml/min/1.73m2 , weak positive rheumatoid factor and cold agglutinins, equivocal Lupus anticoagulant, negative ANCA, clear urinalysis, bilateral chronic inflammatory change on chest xray and thrombi of digital arteries on Doppler ultrasound of hands. She was initially treated for infection due to ongoing temperatures and had multiple scans with no definite source. An inflammatory aetiology was then thought likely due to lack of response to antibiotics and steroid therapy was commenced with settling of fevers and inflammatory markers. Autoimmune screens initially were negative but became more prominent over time with a positive Ro antibody and ultimately a positive Anti PL12 antibody, keeping with anti-synthetase syndrome. She subsequently developed florid interstitial lung disease, further ischemia and ultimately necrosis of her fingertips. Due to the onset of lung disease she was treated with IV steroids, Cyclophosphamide and Prostaglandins with some initial benefit. She received 3 cycles of cyclophosphamide and managed to come off supplemental oxygen. However, she had issues with recurrent chest infections due to immunosuppressive therapy which resulted in delays in her cyclophosphamide pulses and need for antibiotics. She later developed clostridium-difficile gastroenteritis and subsequent ileus of her bowel which was managed conservatively and found it difficult to overcome. As time went on, the progress that she had made with her hands started to deteriorate again. There were also further issues with intestinal obstruction, and sadly ultimately passed away with aspiration pneumonia. Discussion ASS is recognized as a rare autoimmune inflammatory myopathy of unknown etiology, 2–3 times more prevalent in women than in men. The clinical manifestations include myositis, polyarthritis, ILD, mechanic’s hands, and Raynaud phenomenon. The hallmark of ASS is the presence of serum autoantibodies directed against aminoacyl-tRNA synthetases that include Jo-1, PL-7, PL-12, OJ, EJ, KS, Wa, YRS and Zo. Anti-jo1 is the most commonly detected antibody. These autoantibodies may arise after viral infections, or patients may have a genetic predisposition. Our case was interesting as the autoimmune profile was positive for Anti-Ro/SSA and anti-PL-12. Anti-Ro/SSA and anti-La/SSB are traditionally associated with Sjögren’s disease and Sjögren’s-related ILD, however, anti-Ro/SSA has been independently associated with ASS and more severe and fibrotic ILD. It has been described that patients with anti-PL-12-ASS are most often clinically diagnosed with amyopathic dermatomyositis or ILD alone and there is higher prevalence and increased severity of ILD than PM/DM. Moreover, the prevalence of muscle symptoms (weakness and myalgia) is significantly lower in patients with anti-PL7/PL12 as compared to those with anti-Jo1 and less associated with malignancy as compared to DM. Interestingly, anti-PL-12 is also associated with higher rates of Raynaud phenomenon. Our case also reports that not all patients with antisynthetase antibodies or even those classified as ASS have all manifestations of this syndrome. Diagnostic criteria refers to presence of antisynthetase antibody plus two major criteria or one major criterion and two minor criteria (Solomon et all. 2011) or one or more of clinical features (Connors et all.2010). When the lungs are affected, the severity and extent of lung damage generally determines the prognosis because respiratory failure is the leading cause of death. Clinical presentation guides towards therapeutic management that mostly includes corticosteroids, immunosuppressive medications, and/or physical therapy Key learning points Digital ischemia could be a rare presentation of ASS. Clinical features of antisynthetase syndrome (ASS) include interstitial lung disease, ‘mechanic’ hands, myositis, polyarthritis and Raynaud’s phenomenon. Extensive myositis specific antibody testing is strongly recommended even if screening autoimmune serologies are unrevealing. Anti- PL-12 ASS has significantly lower prevalence of muscle symptoms (weakness and myalgia) and more association with Raynaud’s phenomenon as compared to those with anti-Jo1. It is imperative to recognize lesser-known manifestations of ASS in the absence of clinical myositis as delay in diagnosis and treatment worsens the prognosis. Conflicts of interest The authors have declared no conflicts of interest.

scholarly journals AB0092 ANTISYNTHETASE SYNDROME: CLINICAL PROFILE, SEROLOGIC AND TREATMENTS USED IN A COHORT OF PATIENTS FOLLOWED AT THE VIRGEN MACARENA HOSPITAL

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1075.1-1075
Author(s):  
P. Muñoz Reinoso ◽  
I. García Hernández ◽  
M. Ferrer Galván ◽  
F. J. Toyos Sáenz de Miera ◽  
L. Fernández de la Fuente Bursón ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Biological Therapy ◽  
Diagnostic Delay ◽  
Respiratory Function Tests ◽  
Mean Value ◽  
Frequent Pattern ◽  
Antisynthetase Syndrome ◽  
Antisynthetase Antibodies ◽  
Classical Triad

Background:The antisynthetase syndrome (SAS) is characterized by the presence of antisynthetase antibodies, anti-JO1, PL7 y PL12 are the most common; and the classic triad of myositis, arthritis, and diffuse interstitial lung disease (ILD)1. Most patients present incomplete forms and the severity of the ILD determines the prognosis of the disease2.Objectives:to analyze epidemiological, clinical and serological characteristics and treatments used in a cohort of patients with SAS.Methods:descriptive study of review of medical records. Data were collected from 15 patients with SAS followed in the Rheumatology and Pneumology consultations of the Virgen Macarena Hospital (Seville) in the last 10 years. The analysis was carried out using the R software.Results:15 patients were included, 8 men and 7 women. The median age was 56 years (33-77). Seven patients (47%) used to smoke. Four patients (27%) met the classical triad. All of them presented ILD and 8 patients (53%) had arthritis and / or myositis. Five (33%) had mechanic’s hands and six of them (40%) presented Raynaud. Seven (47%) suffered from dyspnea before the SAS diagnosis. The median diagnostic delay was 1 month (0-43). Seven (47%) patients had anti-JO1, 1 (7%) anti-PL7, 2 (13%) anti-PL12 and 2 (13%) patients anti-Ro52. Radiological patterns detected by HRCT were: 5 (33%) NINE, 4 (37%) NIU and 6 (40%) others. The initial treatment included mostly (66%) glucocorticoids (GC) and one or more cFAME. In maintenance, mycophenolate was used in 7 patients (47%), cyclosporine 5 (33%), cyclophosphamide in 3 cases (20%), azathioprine in 3 patients (20%) and methotrexate in 3 of them (20%). Four (37%) patients required a combination of DMARDs and 2 cases needed (13%) biological therapy, Rituximab and Tocilizumab. Changes in the mean value of the initial respiratory function tests (FVC1 and DLCO1) and during follow-up (FVC2 and DLCO2) were not relevant (FVC1 81.5% [42-110], FVC2 81% [59-115]; DLCO1 83% [10-112], DLCO2 80.5% [47-108]). Nine patients (60%) remained clinically stable and 3 patients (20%) progressed radiologically. Four patients died from ILD progression.Conclusion:In this study, the incomplete diagnosis of SAS predominated. The most detected antibody was anti-JO1. ILD is present in all cases, with NINE being the most frequent pattern so multidisciplinary management is necessary. Most used treatments were GC and FAMES combined, some cases required biological therapy.References:[1]Irazoque F, et al. Epidemiology, etiology and classification. Reumatol Clin. 2009;5:2-5.[2]Johnson C, et al. Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type. Respir Med. 2014; 108(10):1542-8.Disclosure of Interests:None declared

scholarly journals SAT0529 CLINICAL CHARACTERISTICS AND TREATMENT PATTERNS IN A PATIENT GROUP WITH INTERSTITIAL LUNG DISEASE.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1222.2-1222
Author(s):  
R. Ortega Castro ◽  
P. S. Laura ◽  
F. U. Pilar ◽  
J. Calvo Gutierrez ◽  
A. Requejo-Jimenez ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Smoking Habit ◽  
Inflammatory Myopathy ◽  
Usual Interstitial Pneumonia ◽  
Treatment Patterns ◽  
Common Symptom ◽  
Radiological Pattern ◽  
Mechanic’S Hands

Background:Diffuse interstitial lung disease (ILD) is frequently associated with connective tissue diseases (CTD) and is one of the main causes of morbidity and mortality in these patients. Recently, the concept of Interstitial Pneumonia with Autoimmune Features (IPAF) has been defined to characterize ILD associated with systemic manifestations limited to subtle serological and clinical autoimmune abnormalities and not fulfilling the international criteria for the diagnosis of a given CTD.Objectives:The objective of this study is to describe the clinical, serological and radiological characteristics, as well as the treatment patterns of patients with ILD referred to a Rheumatology Service for suspected CTDMethods:Observational, cross-sectional study of 43 patients with ILD referred for evaluation to the medical consultation of CTD of the Rheumatology service at the Reina Sofía Hospital. Patients were classified as patients with defined CTD, patient with IPAF and patients with other types of pneumopathy. We conducted a descriptive study of all patients and compared the clinical-analytical-radiological characteristics and treatment patterns of the first two groups.Results:Of the 43 patients, 67.40% were women with a mean age at diagnosis of 65.65 (10.42) years and 53.50% of smoking patientsOf the total of patients, 16 (37.2%) were included in the CTD group, 17 (39.5%) met criteria for IPAF and 10 (23.3%) had another type of pneumopathy.In the CTD group scleroderma was the most frequent disease (6/16), followed by inflammatory myopathy (4/16), Sjögren’s syndrome (3/16), rheumatoid arthritis (2/16) and polymyalgia rheumatic (1/16). In this group of patients, the most common symptom was Raynaud’s phenomenon (RP) (7/16), followed by arthritis (7/16) and mechanic’s hands (3/16). Regarding the most frequently antibodies were ANA (100%), anti-RO (41.7%), anti-citrullinated protein antibodies (30%) and rheumatoid factor (RF) (28.6%).In patients with IPAF, as in the CTD group, the most observed clinical criterion was RP (5/17), followed by arthritis (1/17) and mechanic’s hands (1/17). Among the serological criteria the most common antibodies were ANA (100%), followed by anti-RO (33.3%), anti-RNA synthetase (28.6%) and RF (22.2%).Regarding the radiological pattern, in both groups the most frequent was nonspecific interstitial pneumonia, followed by the indeterminate pattern and usual interstitial pneumonia (UIP) in third place. There were no significant differences by gender and age, between the group of CTD and IPAF, observing in both groups a predominance of women with a similar mean age, being the upper smoking habit in the IPAF group (70.6% vs 31.5%, p= 0.02). Regarding the treatment used, the use of immunosuppressants (IS) was more frequent in CTD group (56.3% vs 11.8%, p = 0.007).Conclusion:The clinical-serological and radiological characteristics were similar among patients with IPAF and CTD, which supports the notion of a similar pathophysiology in both groups. In our cohort patients with CTD received IS more frequently than IPAF group, however, future work would be necessary to assess whether the response to treatment is similar in these populations and if IS can benefit patients with IPAF to long term. In addition, it could be useful to include the UIP pattern within the IPAF classification criteria, not currently included, since it is the third most frequent radiological pattern.References:[1]Respirology, 21 (2016), pp. 245-258[2]Eur Respir J, 46 (2015), pp. 976-987Disclosure of Interests:Rafaela Ortega Castro: None declared, Pérez Sánchez Laura: None declared, Font Ugalde Pilar: None declared, Jerusalem Calvo Gutierrez: None declared, Antonio Requejo-Jimenez: None declared, Simona Espejo-Pérez: None declared, Teresa Gonzalez-Serrano: None declared, María del Carmen Castro Villegas: None declared, Gómez García Ignacio: None declared, Alejandro Escudero Contreras: None declared, Eduardo Collantes Estevez Grant/research support from: ROCHE and Pfizer, Speakers bureau: ROCHE, Lilly, Bristol and Celgene, Maria A Aguirre: None declared

scholarly journals The lungs were on fire: a pilot study of 18F-FDG PET/CT in idiopathic-inflammatory-myopathy-related interstitial lung disease

2021 ◽  
Author(s):  
Junyu Liang ◽  
Heng Cao ◽  
Yinuo Liu ◽  
Bingjue Ye ◽  
Yiduo Sun ◽  
...  
Keyword(s):  
Lymph Node ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Ct Scan ◽  
Mediastinal Lymph Node ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Pet Ct ◽  
Bilateral Lung ◽  
Pet Ct Scan

Abstract Background: Interstitial lung disease (ILD) and its rapid progression (RP) were main contributors to unfavorable outcome of idiopathic inflammatory myopathy (IIM) patients. This study aimed at identifying the clinical value of PET/CT scan in IIM-ILD patients as well as constructing a predicting model for RP-ILD.Methods: Adult IIM-ILD patients who were hospitalized at four divisions of the First Affiliated Hospital, Zhejiang University School of Medicine (FAHZJU) from Jannuary 1st 2017 to December 31st 2020 were reviewed. PET/CT scan as well as other factors of patients who met the inclusion and exclusion criteria were collected and analyzed.Results: A total of 61 IIM-ILD patients were finally enrolled into this study. Twenty-one patients (34.4%) developed RP-ILD and 24 patients (39.3%) died in follow-up. After false discovery rate (FDR) correction, percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%, P=0.014), bilateral lung mean standard uptake value (SUVmean, P=0.014) and abnormal mediastinal lymph node (P=0.045) were significantly different in comparison between RP-ILD and non-RP-ILD groups. A “DLM” model was hereby established by including the above three values to predict RP-ILD with a cutoff value of ≥2 and an area under the curve (AUC) of 0.905. Higher bilateral lung SUVmean (P=0.019) and spleen SUVmean (P=0.011) were observed in IIM-ILD patients who died within three months, and a moderate correlation was recognized between the two values. Conclusions: Elevated bilateral lung SUVmean and abnormal mediastinal lymph node were associated with RP-ILD in IIM-ILD patients. The “DLM” model was valuable in predicting RP-ILD and demanded further evaluation.

ADAM-17 is expressed in the inflammatory myopathy and is involved with interstitial lung disease

2018 ◽  
Vol 37 (4) ◽  
pp. 1017-1024 ◽  
Author(s):  
Airi Nishimi ◽  
Takeo Isozaki ◽  
Shinichiro Nishimi ◽  
Sho Ishii ◽  
Takahiro Tokunaga ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy

scholarly journals Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America

Rheumatology ◽  
2020 ◽  
Author(s):  
Sara E Sabbagh ◽  
Jessica Neely ◽  
Albert Chow ◽  
Marietta DeGuzman ◽  
Jamie Lai ◽  
...  
Keyword(s):  
Risk Factors ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
Inflammatory Myopathy ◽  
Severe Infection ◽  
Idiopathic Inflammatory Myopathy ◽  
Pneumocystis Jirovecii ◽  
Pneumocystis Jirovecii Pneumonia ◽  
The Usa

Abstract Objectives Pneumocystis jirovecii pneumonia (PJP) is associated with significant morbidity and mortality in adult myositis patients; however, there are few studies examining PJP in juvenile myositis [juvenile idiopathic inflammatory myopathy (JIIM)]. The purpose of this study was to determine the risk factors and clinical phenotypes associated with PJP in JIIM. Methods An research electronic data capture (REDCap) questionnaire regarding myositis features, disease course, medications and PJP infection characteristics was completed by treating physicians for 13 JIIM patients who developed PJP (PJP+) from the USA and Canada. Myositis features and medications were compared with 147 JIIM patients without PJP (PJP–) from similar geographic regions who enrolled in National Institutes of Health natural history studies. Results PJP+ patients were more often of Asian ancestry than PJP– patients [odds ratio (OR) 8.7; 95% CI 1.3, 57.9]. Anti- melanoma differentiation associated protein 5 (MDA5) autoantibodies (OR 12.5; 95% CI 3.0, 52.4), digital infarcts (OR 43.8; 95% CI 4.2, 460.2), skin ulcerations (OR 12.0; 95% CI 3.5, 41.2) and interstitial lung disease (OR 10.6; 95% CI 2.1, 53.9) were more frequent in PJP+ patients. Before PJP diagnosis, patients more frequently received pulse steroids, rituximab and more immunosuppressive therapy compared with PJP– patients. Seven PJP+ patients were admitted to the intensive care unit and four patients died due to PJP or its complications. Conclusions PJP is a severe infection in JIIM that can be associated with mortality. Having PJP was associated with more immunosuppressive therapy, anti-MDA5 autoantibodies, Asian race and certain clinical features, including digital infarcts, cutaneous ulcerations and interstitial lung disease. Prophylaxis for PJP should be considered in juvenile myositis patients with these features.

Prognosis of adult idiopathic inflammatory myopathy-associated interstitial lung disease: a retrospective study of 679 adult cases

Rheumatology ◽  
2020 ◽  
Author(s):  
Shan Li ◽  
Yuxin Sun ◽  
Chi Shao ◽  
Hui Huang ◽  
Qian Wang ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Retrospective Study ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Mortality Rates ◽  
Trna Synthetase ◽  
Significant Difference ◽  
All Cause Mortality

Abstract Objectives Few studies have investigated the prognostic factors for idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) across different clinical/serological phenotypes. Methods We conducted a retrospective analysis of patients diagnosed with IIM between January 2012 and December 2017. Results Of the 760 IIM cases registered, 679 adult cases were included in this study. ILD was present in 508 cases, and the presence of ILD in the clinically amyopathic DM, DM and PM groups was 92.7, 73.6 and 55.1%, respectively (P < 0.01). The prevalence of ILD in the anti-synthetase antibody (ASA)+-IIM group was higher than that in ASA–-IIM group (95.2 vs 72.4%, P < 0.01); no such difference was found between the anti-histidyl-tRNA synthetase (Jo-1)+-IIM and Jo-1–ASA+-IIM groups (93.0 vs 98.5%, P > 0.05). The prevalence of ILD in the melanoma differentiation-associated protein-5 (MDA-5)+-IIM group was higher than that in MDA-5–-IIM group (97.8 vs 72.1%, P < 0.01). Among adults with IIM, men with concurrent ILD, who were older than 50 years, were most likely to die. No significant difference was found in the all-cause mortality rates between DM-ILD and clinically amyopathic DM-ILD groups (33.3 vs 23%, P > 0.05), although both were higher than that in PM group (13.2%, P = 0.01 and P < 0.05, respectively). No difference was found in the all-cause mortality rates between MDA5–ASA–-IM-ILD and MDA5–ASA+-IM-ILD groups (17.2 vs 12.8%, P > 0.05), and both were lower than that in MDA5+ASA–-IM-ILD group (33.7%, P < 0.05). Conclusion The prevalence of ILD in IIM and the prognosis of IIM-ILD patients may vary depending on the statuses of the ASA and MDA-5 antibodies.

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