Dataset Information

Antisynthetase syndrome and interstitial lung disease: A case report.

ABSTRACT:

Introduction

Myositis, Raynaud's phenomenon, fever, interstitial lung disease, mechanic's hands, and arthropathy are symptoms of Antisynthetase Syndrome (ASS), which is defined by the development of antibodies against t-ribonucleic acid (RNA) synthetase, particularly anti-Jo-1.

Case presentation

The case is about 29 years female with 1 month history of non-productive cough and dyspnea on exertion which was later diagnosed as ASS.

Discussion

The diagnosis of an inflammatory myopathy is based on clinical findings such as subacute development of symmetrical muscle weakness and signs such as laboratory investigations revealing skeletal muscle inflammation. Creatinine phosphokinase (CPK) is mainly used to demonstrate skeletal muscle involvement.

Conclusion

Interstitial lung disease is a frequent occurrence and is associated with a bad prognosis during the course of antisynthetase syndrome.

SUBMITTER: Dahal K

PROVIDER: S-EPMC9577423 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature

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Publications

Antisynthetase syndrome and interstitial lung disease: A case report.

Dahal Krishna K Chaudhary Anand A Rawal Laba L Ray Umesh U Paudel Sandip S Khanal Pitambar P Gyawali Pawan P Sah Sanjit Kumar SK Shrestha Kishan Kumar KK Pandey Shailendra S

Annals of medicine and surgery (2012) 20220908

<h4>Introduction</h4>Myositis, Raynaud's phenomenon, fever, interstitial lung disease, mechanic's hands, and arthropathy are symptoms of Antisynthetase Syndrome (ASS), which is defined by the development of antibodies against t-ribonucleic acid (RNA) synthetase, particularly anti-Jo-1.<h4>Case presentation</h4>The case is about 29 years female with 1 month history of non-productive cough and dyspnea on exertion which was later diagnosed as ASS.<h4>Discussion</h4>The diagnosis of an inflammatory ...[more]

PMID: 36268301

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Project description:ImportanceAutoimmune disorders can affect various organs and if refractory, can be life threatening. Recently, CD19-targeting-chimeric antigen receptor (CAR) T cells were efficacious as an immune suppressive agent in 6 patients with refractory systemic lupus erythematosus and in 1 patient with antisynthetase syndrome.ObjectiveTo test the safety and efficacy of CD19-targeting CAR T cells in a patient with severe antisynthetase syndrome, a complex autoimmune disorder with evidence for B- and T-cell involvement.Design, setting, and participantsThis case report describes a patient with antisynthetase syndrome with progressive myositis and interstitial lung disease refractory to available therapies (including rituximab and azathioprine), who was treated with CD19-targeting CAR T cells in June 2022 at University Hospital Tübingen in Tübingen, Germany, with the last follow-up in February 2023. Mycophenolate mofetil was added to the treatment to cotarget CD8+ T cells, hypothesized to contribute to disease activity.ExposurePrior to treatment with CD19-targeting CAR T cells, the patient received conditioning therapy with fludarabine (25 mg/m2 [5 days before until 3 days before]) and cyclophosphamide (1000 mg/m2 [3 days before]) followed by infusion of CAR T cells (1.23×106/kg [manufactured by transduction of autologous T cells with a CD19 lentiviral vector and amplification in the CliniMACS Prodigy system]) and mycophenolate mofetil (2 g/d) 35 days after CD19-targeting CAR T-cell infusion.Main outcomes and measuresThe patient's response to therapy was followed by magnetic resonance imaging of the thigh muscle, Physician Global Assessment, functional muscle and pulmonary tests, and peripheral blood quantification of anti-Jo-1 antibody levels, lymphocyte subsets, immunoglobulins, and serological muscle enzymes.ResultsRapid clinical improvement was observed after CD19-targeting CAR T-cell infusion. Eight months after treatment, the patient's scores on the Physician Global Assessment and muscle and pulmonary function tests improved, and there were no detectable signs of myositis on magnetic resonance imaging. Serological muscle enzymes (alanine aminotransferase, aspartate aminotransferase, creatinine kinase, and lactate dehydrogenase), CD8+ T-cell subsets, and inflammatory cytokine secretion in the peripheral blood mononuclear cells (interferon gamma, interleukin 1 [IL-1], IL-6, and IL-13) were all normalized. Further, there was a reduction in anti-Jo-1 antibody levels and a partial recovery of IgA (to 67% of normal value), IgG (to 87%), and IgM (to 58%).Conclusions and relevanceCD19-targeting CAR T cells directed against B cells and plasmablasts deeply reset B-cell immunity. 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.

Project description:ObjectivesTo explore the association between the extent of CT abnormalities by quantitative imaging analysis (QIA) and clinical/physiological disease parameters in patients with antisynthetase syndrome associated interstitial lung disease (ARS-ILD).MethodsWe analysed 20 patients with antisynthetase antibodies and active ILD enrolled in the Abatacept in Myositis-Associated Interstitial Lung Disease study. High-resolution chest CT was obtained at weeks 0, 24 and 48 and QIA scored the extent of ground glass (quantitative score for ground glass), fibrosis (quantitative score for lung fibrosis, QLF) and total ILD (quantitative ILD, QILD). Mixed-effects models estimated longitudinal QIA scores over time. Associations between QIA scores with clinical/physiological parameters were analysed longitudinally using repeated-measures mixed-effects models.ResultsPatients were median age 57 years, 55% males and 85% white. Higher (worse) baseline QIA scores correlated with lower baseline forced vital capacity (FVC) and diffusing capacity adjusted for haemoglobin (DLCO). Longitudinal QIA trajectories trended towards improving scores during the trial, and patients on O2 at baseline had worsening QIA trajectories which were different from patients who were not on O2. Longitudinal QIA scores demonstrated strong associations with both FVC and DLCO over time. Higher QILD scores over time were also associated with worse dyspnoea scores, pulmonary visual analogue scale, physician and patient global disease activity, health status in 6/8 domains of the Short Form-36 and higher oxygen requirements. Patients with significant radiographic improvement at 48 weeks had higher baseline QLF, QILD and worse DLCO.ConclusionsLongitudinal QIA scores associate with lung physiology, patient perception of respiratory status, overall disease activity and quality of life over time in ARS-ILD. QIA may allow reproducible monitoring of disease progression and response to therapy over time.Trial registration numberNCT03215927.

Dataset Information

Antisynthetase syndrome and interstitial lung disease: A case report.

Introduction

Case presentation

Discussion

Conclusion

Publications

Antisynthetase syndrome and interstitial lung disease: A case report.

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