Co-Infection with Nocardia Terpene and Pneumocystis Jirovecii in a Patient with Anti-Synthetase Syndrome: A Case Report.

Background: Pulmonary infection is a common clinical complication associated with glucocorticoid. There have been no reported cases of mixed infections involving Nocardia and Pneumocystis jirovecii combined with anti-synthetase syndrome (ASS) activity. Methods: This study conducted a retrospective analysis of the clinical data from a patient with active ASS, treated for a pulmonary coinfection. Results: The patient exhibited fever, asthma, and cough as initial symptoms. Chest CT scan revealed multiple infiltration shadows, consolidation shadows, nodules, mass shadows, and internal cavities in both lungs. BALF mNGS detected Nocardia terpene and Pneumocystis jiroveci. Treatment with sulfamethoxazole/trimethoprim and corticosteroids led to an improvement. However, the patient experienced recurrent fever and a new rash with the reduction of the glucocorticoid dosage. Further investigation identified positive anti-Jo-1 and anti-Ro-52 antibodies and myogenic lesions on electromyography, which confirmed the diagnosis of ASS. Following treatment with immunoglobulin, methylprednisolone, and cyclosporine, the patient's condition significantly improved. Conclusion: Immunodeficiency patients are susceptible to opportunistic infections. mNGS is valuable for diagnosis and treatment. Although the image of Nocardia terpene and Pneumocystis jiroveci infections lack specificity, they exhibit distinctive features. Should fever and skin lesions reoccur post-effective anti-infective therapy, it is imperative to explore non-infectious causes and expedite autoantibody testing.

Diffuse Interstitial Lung Disease Revealing Antisynthetase Syndrome.

Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases. They may be revelatory of the disease or occur during follow-up. Antisynthetase syndrome (ASS) is a complex and heterogeneous autoimmune disorder. Antisynthetase antibodies, in particular the anti-Jo-1 antibody, characterize this syndrome. The occurrence and severity of ILD determine the prognosis, which in turn determines therapeutic management. We report the case of a 53-year-old female patient presenting with ILD, revealing the diagnosis of ASS. The evolution was favorable with bolus corticosteroids associated with cyclophosphamide.

Clinical and prognostic associations of anti-Jo-1 antibody levels in patients with antisynthetase syndrome.

OBJECTIVE: To investigate the association of serum anti-Jo-1 antibody levels with the disease activity and prognosis in anti-Jo-1-positive patients with antisynthetase syndrome (ASS). METHODS: This study included 115 anti-Jo-1-positive patients with ASS who were admitted to China-Japan Friendship Hospital between 2009 and 2019. Anti-Jo-1 antibody serum levels at initial admission and follow-up were determined by enzyme-linked immunosorbent assay (ELISA). Global and organ disease activity was assessed at baseline and follow-up according to the International Myositis Assessment and Clinical Studies guidelines. RESULTS: Among enrolled patients, 70 (60.9%) patients initially presented with interstitial lung disease (ILD), and 46 (40%) patients presented with with muscle weakness at initial admission. At baseline, patients with ILD had lower levels of anti-Jo-1 antibodies than those without ILD (p = 0.012). Baseline anti-Jo-1 antibody levels were higher in patients with muscle weakness, skin involvement, and arthritis (all p < 0.05) compared to those without these manifestations. Baseline anti-Jo-1 antibody levels were positively correlated with skin visual analogue scale (VAS) scores (r = 0.25, p = 0.006), but not with disease activity in other organs. However, changes in anti-Jo-1 antibody levels were significantly positively correlated with the changes in PGA (ß = 0.002, p = 0.001), muscle (ß = 0.003, p < 0.0001), and pulmonary (ß = 0.002, p = 0.013) VAS scores, but not with skin and joint VAS scores. Older age of onset (hazard ratio [HR] 1.069, 95% confidence interval [CI]:1.010-1.133, p = 0.022) and higher C-reactive protein (CRP) levels (HR 1.333, 95% CI: 1.035-1.717, p = 0.026) were risk factors for death. CONCLUSION: Anti-Jo-1 titers appear to correlate more with disease activity changes over time rather than with organ involvement at baseline, which provides better clinical guidance for assessing the disease course using anti-Jo-1 levels.

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease.

Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud's phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions.

Clinical evolution of antisynthetase syndrome-associated interstitial lung disease after COVID-19 in a man with Klinefelter syndrome: A case report.

BACKGROUND: This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome (AS) following coronavirus disease 2019 (COVID-19) in a 33-year-old man diagnosed with Klinefelter syndrome (KS). CASE SUMMARY: A 33-year-old man with a diagnosis of KS was admitted to the Department of Pulmonary and Critical Care Medicine of a tertiary hospital in China for fever and shortness of breath 2 wk after the onset of COVID-19. Computed tomography of both lungs revealed diffuse multiple patchy heightened shadows in both lungs, accompanied by signs of partial bronchial inflation. Metagenomic next-generation sequencing of the bronchoalveolar lavage fluid suggested absence of pathogen. A biopsy specimen revealed organizing pneumonia with alveolar septal thickening. Additionally, extensive auto-antibody tests showed strong positivity for anti-SSA, anti-SSB, anti-Jo-1, and anti-Ro-52. Following multidisciplinary discussions, the patient received a final diagnosis of AS, leading to rapidly progressing respiratory failure. CONCLUSION: This study underscores the clinical progression of AS-associated interstitial lung disease subsequent to viral infections such as COVID-19 in patients diagnosed with KS.

Quick response to rituximab in a patient with Ro52-Jo1 complex-associated antisynthetase syndrome debuting with rapidly progressive interstitial lung disease and diffuse alveolar haemorrhage / Rápida respuesta a rituximab en un paciente con síndrome antisintetasa asociado a anti-Ro52-Jo1 que debuta con enfermedad intersticial rápidamente progresiva y hemorragia alveolar difusa

Antisynthetase syndrome (AAS) is an inflammatory myopathy that may debut with interstitial lung disease (ILD). A few studies show cases in which patients with high concentrations of anti-Ro52 in patients with ILD might achieve a better response if treated with rituximab than other patients with ILD and Ro-52 whose levels are lower. We present a patient with lung involvement as the first and unique manifestation of AAS with anti-Jo and anti-Ro52-positive antibodies. The ILD was rapid and progressive and led him into the intensive care unit in a matter of days, despite several immunosuppressive treatments. As soon as the patient received this treatment, he experienced an improvement. We therefore suggest the prompt determination of anti-Ro52 antibody concentrations in this subgroup of patients, which would offer a therapeutic approach based first on rituximab over other immunosuppressive treatments (AU)

El síndrome antisintetasa (SA) es una miopatía inflamatoria que puede debutar en forma de enfermedad pulmonar intersticial (EPI). Algunos estudios muestran casos de pacientes con EPI secundaria a miopatía inflamatoria y positividad de anti-Ro52 a altas concentraciones que presentan mejor respuesta a rituximab que pacientes similares con positividad de anti-Ro52 a bajas concentraciones. Presentamos un paciente con enfermedad intersticial como primera y única manifestación de SA y con positividad para anti-Ro52 a concentraciones altas y anti-Jo1. La EPI fue rápida y progresiva, conduciéndole a ingreso en la unidad de cuidados intensivos e intubación orotraqueal a pesar de varios tratamientos inmunosupresores. Solo experimentó mejoría cuando se inició rituximab. Por ello, proponemos medir los títulos del anticuerpo contra Ro-52 en todos los pacientes con EPI secundaria a SA y positividad de anti-Ro52, y si se evidencian valores altos decidir rituximab como tratamiento de primera línea antes que otras terapias inmunosupresoras (AU)

Antisynthetase Syndrome With Predominant Pulmonary Involvement: A Case Report.

Antisynthetase syndrome (ASyS) is an autoimmune disease characterized by the presence of aminoacyl-transfer RNA synthetase antibodies. Its clinical presentation is variable and may include interstitial lung disease (ILD), myositis, arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." ILD is more prevalent in this entity when compared to other idiopathic inflammatory myopathies and imparts greater severity to the condition. Here, we report the case of a 42-year-old female patient who sought care for severe ILD and persistent fever. Her diagnosis was made only after the detection of anti-Jo1 autoantibodies. Treatment was refractory to both prednisone monotherapy and cyclophosphamide pulse therapy, requiring the introduction of rituximab. A high degree of clinical suspicion is required to allow early diagnosis of ASyS in patients with pulmonary involvement in the absence of accompanying muscle weakness or other clinical symptoms.

Antisynthetase Syndrome: The Classical Phenotype With a Twist.

Antisynthetase syndrome is a systemic autoimmune rheumatic disease characterized by multiple organ involvement, including interstitial lung disease, myositis, non-erosive arthritis, fever, Raynaud's phenomenon, "mechanic's hands," and the presence of autoantibodies against aminoacyl-tRNA synthetases, mainly anti-Jo1 (histidyl) antibodies. Patients with antisynthetase syndrome and active muscle inflammation are usually presented with elevated creatine phosphokinase levels, even in the range of acute rhabdomyolysis. Despite that, the presence of myoglobinuric acute kidney injury is rarely seen in patients with myositis-associated rhabdomyolysis. Herein, we report the case of a 64-year-old man who presented with acute kidney injury due to severe rhabdomyolysis in the setting of antisynthetase syndrome diagnosed by the classical clinical triad of (1) interstitial lung disease, (2) non-erosive arthritis, and (3) active myositis and the presence of anti-Jo1 antibodies. The diagnosis was confirmed by muscle biopsy histological findings as well as electromyography. In this case report, we also discuss the classical clinical manifestations of antisynthetase syndrome and a twist toward this unusual complication associated with active muscle inflammation.

Anti-Jo-1 autoantibodies: biomarkers of severity and evolution of the disease in antisynthetase syndrome.

BACKGROUND: Anti-Jo-1 autoantibodies represent essential markers in the diagnosis of antisynthetase syndrome (ASS). In this retrospective study, we aimed to investigate whether their concentrations and fluctuations could both respectively reflect the severity and evolution of ASS. METHODS: Between 2015 and 2020, clinical and biological features of ASS patients with at least one positive measure of anti-Jo-1 autoantibody were collected. At each serum sampling, we assessed myositis activity by using the Myositis Intention to Treat Activities Index (MITAX) and compared anti-Jo-1 concentrations with ASS severity, anti-Jo-1 concentrations between patients with and without active disease, and changes in anti-Jo-1 concentrations with disease activity. RESULTS: Forty-eight patients with ASS had at least one positive determination of anti-Jo-1 concentration. Among them, twenty-nine patients had at least two determinations of anti-Jo-1 autoantibody in their follow-up. We showed that these autoantibody concentrations were significantly correlated with MITAX (r = 0.4, p = 0.03) and creatine kinase concentration (r = 0.34, p = 0.002) and that they were significantly higher in patients with active disease than in those with inactive disease (91.7 IU/L vs 44.4 IU/L, p = 0.016). During follow-up, we found a significant correlation between fluctuations of anti-Jo-1 autoantibody concentrations and MITAX score (r = 0.7, p < 0.0001). CONCLUSION: Our results suggest that anti-Jo-1 autoantibody concentration could be a predictive marker of the severity and evolution of ASS and show that their quantification could represent a precious tool for disease monitoring and for improving the therapeutic management of ASS patients.

A Case of Antisynthetase Syndrome in the Setting of SARS-Cov-2 Infection.

Antisynthetase syndrome is a complex autoimmune disorder, and one of the key criteria for diagnosis is the presence of myositis. Additionally, evidence of interstitial lung disease (ILD) is another important indicator for diagnosis; other clinical features associated with antisynthetase syndrome include arthritis, unexplained and persistent fever, Raynaud's phenomenon, and the presence of mechanic's hands. We report a case of a 36-year-old male who presented to the emergency department with shortness of breath and proximal muscle weakness in the setting of severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection, as his inflammatory markers were elevated and he exhibited features suspicious for antisynthetase syndrome, he was started on methylprednisolone 40 mg intravenously every eight hours, and a myositis panel was checked. In addition, a chest computed tomography (CT) exhibited ground-glass opacities which were compatible with coronavirus disease 2019 (COVID-19). A magnetic resonance image (MRI) of both thighs was done, revealing significant swelling and confirming the suspicion of myositis as his muscle strength in his lower extremities took significant time to improve. As days passed, his muscle strength improved significantly and his creatine phosphatase kinase (CPK) values trended down, indicating that his myositis was improving as well. He was transitioned to oral prednisone 60 mg daily and was discharged home with a rheumatology follow-up to define long-term treatment. A myositis panel revealed anti-glycyl-transferRNA synthetase (EJ) autoantibody positivity and a diagnosis was established. Our case revealed how sometimes laboratory values do not necessarily correlate with disease severity and how we have to do a thorough history of present illness and physical exam to think about unusual diagnoses before putting laboratory data into context.