Antisynthetase syndrome

A chronic AUTOIMMUNE DISEASE that affects the muscles and other parts of the body. The signs and symptoms may include muscle inflammation (myositis), POLYARTHRITIS; INTERSTITIAL LUNG DISEASES; thickening and cracking of the hands, and RAYNAUD PHENOMENON. it may be caused by AUTOANTIBODIES against AMINO ACYL-TRNA SYNTHETASES.

Networked: 251 relevant articles (7 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Myositis (Idiopathic Inflammatory Myopathies)
2.	Dermatomyositis (Dermatopolymyositis)
3.	Muscular Diseases (Myopathy)
4.	Inclusion Body Myositis
5.	Joint Diseases (Joint Disease)

Experts

1.	Benveniste, Olivier: 10 articles (01/2021 - 09/2012)
2.	Hervier, Baptiste: 10 articles (12/2020 - 10/2009)
3.	Cavagna, Lorenzo: 9 articles (01/2022 - 09/2010)
4.	Lundberg, Ingrid E: 8 articles (12/2021 - 11/2010)
5.	Mammen, Andrew L: 6 articles (01/2022 - 11/2014)
6.	Montecucco, Carlomaurizio: 6 articles (01/2022 - 09/2010)
7.	Hachulla, Eric: 6 articles (01/2021 - 12/2012)
8.	Meyer, Alain: 6 articles (11/2019 - 12/2012)
9.	Christopher-Stine, Lisa: 5 articles (12/2021 - 06/2009)
10.	Caporali, Roberto: 5 articles (11/2019 - 09/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Antisynthetase syndrome:

1.	SteroidsIBA 03/04/2017 - "Taken together, these observations suggest that administration of immunoglobulin subcutaneously may be a useful therapeutic approach to tackle steroid-refractory antisynthetase syndrome while ensuring minimal side effects and improved treatment compliance. " 10/01/2018 - "Management The diagnosis of antisynthetase syndrome was made and the patient was treated with steroids and azathioprine with a good response. " 04/01/2014 - "Steroids remain the first-line treatment for myositis-associated ILD and the antisynthetase syndrome, though other traditional immunosuppressants have demonstrated efficacy in numerous studies. "
2.	Mycophenolic Acid (Cellcept)FDA LinkGeneric 01/01/2023 - "Together with mycophenolate mofetil, CD19-targeting CAR T cells may break pathologic B-cell, as well as T-cell responses, inducing remission in refractory antisynthetase syndrome." 10/01/2009 - "Long-term efficacy of mycophenolate mofetil in a case of refractory antisynthetase syndrome." 05/01/2007 - "[Antisynthetase syndrome with good response to mycophenolate mofetil]." 01/01/2023 - "He was diagnosed with antisynthetase syndrome and treated with high dose corticosteroids and mycophenolate mofetil with a fair response." 09/01/2020 - "Good response to mycophenolate mofetil on treatment of interstitial lung disease in polymyositis associated with antisynthetase syndrome positive for anti-EJ and anti-Ro52 antibodies."
3.	Immunoglobulins (Immunoglobulin)IBA 03/04/2017 - "Administration of immunoglobulins intravenously is recommended in patients with drug-resistant antisynthetase syndrome. " 03/04/2017 - "Subcutaneous immunoglobulins for the treatment of a patient with antisynthetase syndrome and secondary chronic immunodeficiency after anti-CD20 treatment: a case report." 07/01/2019 - "Long-term treatment with human immunoglobulin for antisynthetase syndrome-associated interstitial lung disease." 03/04/2017 - "She is the first case of a patient with multidrug-resistant antisynthetase syndrome featuring pulmonary involvement and arthropathy, and chronic secondary immune deficiency with recurrent infections, after anti-CD20 treatment, in which her primary antisynthetase syndrome-related symptoms and secondary immune deficiency were treated successfully with subcutaneous administration of immunoglobulin. " 03/04/2017 - "Taken together, these observations suggest that administration of immunoglobulin subcutaneously may be a useful therapeutic approach to tackle steroid-refractory antisynthetase syndrome while ensuring minimal side effects and improved treatment compliance. "
4.	AntibodiesIBA 03/01/2022 - "This study aimed to diagnose and treat a patient with anti-Ro52-positive antisynthetase syndrome (ASS), investigate the association between anti-Ro52 antibodies and ASS, and determine its clinical significance. " 01/01/2017 - "Our study investigates the clinical phenotypes and outcomes of patients with antisynthetase syndrome with distinct antisynthetase antibodies and highlights the link between the anti-PL7 antibody and RP-ILD." 04/01/2014 - "Two recent studies showed that patients with an antisynthetase syndrome associated with anti-Jo1 antibodies have more severe prognosis than antisynthetase syndrome associated with other antibodies (i.e. " 06/01/2023 - "Antisynthetase Syndrome With Paraneoplastic Antibodies." 01/01/2023 - "Sixty-six percent of antisynthetase syndrome patients had 'chronic ILD' compared with the more rapidly progressive ILD (RP-ILD) seen in 69% of patients with anti-MDA5 antibodies. "
5.	Rituximab (Mabthera)FDA Link 03/01/2012 - "Efficacy of rituximab in refractory antisynthetase syndrome." 01/01/2016 - "Rituximab (RTX) has been used successfully for the treatment of severe Jo1 antibody-associated antisynthetase syndrome. " 01/01/2016 - "Rituximab in the Treatment of Jo1 Antibody-associated Antisynthetase Syndrome: Anti-Ro52 Positivity as a Marker for Severity and Treatment Response." 01/01/2015 - "Treatment with rituximab for antisynthetase syndrome itself plays a fundamental role in the development of infectious complications. " 01/01/2015 - "We herein present the case of a 71-year-old man who was diagnosed with antisynthetase syndrome and treated with rituximab. "
6.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2013 - "CYC is effective and substantially safe in patients with anti-Jo1 antisynthetase syndrome with corticosteroid-refractory ILD. " 03/01/2002 - "We report five cases of antisynthetase syndrome with particular clinical features and good response to corticosteroids. " 01/01/2023 - "He was diagnosed with antisynthetase syndrome and treated with high dose corticosteroids and mycophenolate mofetil with a fair response." 01/01/2022 - "A patient with antisynthetase syndrome with pulmonary and muscular involvement was treated with immunosuppressive agents without corticosteroids. " 05/01/2015 - "A 75-year-old Japanese woman who had been treated for 17 years with a corticosteroid for antisynthetase syndrome with antithreonyl-tRNA synthetase antibody developed swelling of her right elbow. "
7.	Immunosuppressive Agents (Immunosuppressants)IBA 07/01/2018 - "This article presents a clinical case of antisynthetase syndrome with severe ARDS successfully treated with immunosuppressive agents and ECMO. " 01/01/2022 - "A patient with antisynthetase syndrome with pulmonary and muscular involvement was treated with immunosuppressive agents without corticosteroids. " 04/01/2014 - "Steroids remain the first-line treatment for myositis-associated ILD and the antisynthetase syndrome, though other traditional immunosuppressants have demonstrated efficacy in numerous studies. " 01/01/2022 - "Antisynthetase syndrome associated with interstitial lung disease (ILD) and myopathy can be successfully treated without corticosteroids.A combination of several immunosuppressive agents can be a therapeutic alternative in case of refractory ILD."
8.	AutoantibodiesIBA 01/01/2023 - "Phenotypic Profiles Among 72 Caucasian and Afro-Caribbean Patients with Antisynthetase Syndrome Involving Anti-PL7 or Anti-PL12 Autoantibodies." 01/01/2023 - "Anti-Jo-1 autoantibodies represent essential markers in the diagnosis of antisynthetase syndrome (ASS). " 01/01/2021 - "This autoantibody was associated with an antisynthetase syndrome phenotype and might predict clinical outcomes in patients with definite DM." 01/01/2020 - "These findings, combined with the presence of anti-Jo-1 autoantibodies in BAL fluid and GC-like structures in the lungs, suggest that immune activation against HisRS might take place within the lungs of patients with IIM/antisynthetase syndrome." 01/01/2020 - "Anti-Jo-1 autoantibodies were detected in BAL fluid and germinal center (GC)-like structures were seen in the lung biopsy samples from patients with IIM/antisynthetase syndrome. "
9.	Jo-1 antibodyIBA 08/29/2018 - "Subsequently, an autoimmune screen was positive for anti-Jo-1 antibody associated with antisynthetase syndrome. " 07/01/2016 - "We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. " 06/01/2008 - "Combined interstitial lung disease and the presence of 'mechanic's hands' could be a clinical clue in the early diagnosis of the rare disease, amyopathic antisynthetase syndrome for which anti-Jo-1 antibody is a useful diagnostic tool. " 01/01/2022 - "Antisynthetase syndrome (ASS) or anti-Jo-1 antibody syndrome has a classic clinical presentation including arthritis, myositis, interstitial lung disease, mechanic hands, and/or Raynaud's phenomenon. " 04/10/2016 - "In idiopathic inflammatory myopathies, the presence of anti-Jo-1 antibody defines a distinct clinical phenotype (myositis, arthritis, interstitial lung disease, Raynaud's phenomenon fever, mechanic's hands), called antisynthetase syndrome. "
10.	Azathioprine (Imuran)FDA LinkGeneric 04/01/2017 - "We report a case of EMN in a 50-year-old man undergoing azathioprine therapy for antisynthetase syndrome." 07/07/2022 - "A 25-Year-Old Saudi Woman with a 2-Year History of Antisynthetase Syndrome with Interstitial Lung Disease Who Commenced Azathioprine Treatment in the Third Trimester of Pregnancy and Had a Successful Birth at Term." 04/01/2017 - "Eruptive melanocytic nevi during azathioprine therapy for antisynthetase syndrome." 10/01/2017 - "Her medical history was remarkable for recent radiation therapy for recurrent breast cancer and antisynthetase syndrome complicated by interstitial lung disease and controlled with azathioprine. " 09/01/2019 - "Efficacy and adverse effects of methotrexate compared with azathioprine in the antisynthetase syndrome."

Therapies and Procedures

1.	Therapeutics 03/01/2012 - "The rationale for the use of B-cell depleting therapies in antisynthetase syndrome has been explored in light of the current understanding of the pathogenesis of this condition." 01/01/2019 - "Background and objective: To describe the clinical characteristics including the bronchoalveolar lavage fluid (BALF) characteristics of patients with antisynthetase syndrome (AS) associated interstitial lung disease (ILD) in a tertiary ILD outpatient clinic, their medical therapy and outcome. " 08/01/1996 - "The "antisynthetase syndrome" may occur in the absence of clinical myositis, and the ILD in these patients is usually responsive to therapy. " 03/01/2011 - "Although various autoimmune phenomena, including lupus-like diseases, vasculitides, or psoriatic skin lesions, are associated with antitumor necrosis factor (TNF) therapy, the development of PM/dermatomyositis (DM) or antisynthetase syndrome following anti-TNF therapy is extremely rare. " 09/01/2012 - "Our study outlines the burden of chest involvement for the prognosis of antisynthetase syndrome in terms of patients' requirement for DMARD therapy."
2.	Lung Transplantation 12/01/2019 - "Bilateral lung transplantation in antisynthetase syndrome." 08/01/2013 - "A 13-year-old with interstitial lung disease due to Antisynthetase Syndrome and pulmonary arterial hypertension underwent emergent bilateral lung transplantation after a rapid clinical decline. " 10/01/2016 - "A composite endpoint was defined, comprising death due to respiratory failure directly related to antisynthetase syndrome (ASS), the need for long-term oxygen therapy or lung transplantation. "
3.	Plasma Exchange 12/01/2015 - "Therapeutic plasma exchange in antisynthetase syndrome with severe interstitial lung disease." 01/01/2023 - "We report the case of a 42-year-old female suffering from severe iTTP associated with anti-Jo-1 positive antisynthetase syndrome, which was successfully treated with combination therapy of intravenous immune globulin, rituximab and plasma exchange. "
4.	Cell- and Tissue-Based Therapy (Cell Therapy) 01/01/2014 - "[Use of anti-B-cell therapy in case of antisynthetase syndrome as the severest subtype of polymyositis/dermatomyositis]."
5.	Extracorporeal Membrane Oxygenation 07/01/2018 - "The subsequent diagnosis of antisynthetase syndrome in patients with acute respiratory distress syndrome (ARDS) is unusual, even more so when a veino-veinous (VV) extracorporeal membrane oxygenation (ECMO) is required. "