Search

Yinying Li,1 Qiuming Li,2 Haihua Lei,3 Xiaorong Wei,4 Tao Feng,2 Huajiao Qin,2 Hongchun Huang,2 Minchao Duan1,2 1The Second Clinical Medical College, Guangxi Medical University, Nanning, Guangxi, 530021, People’s Republic of China; 2Department of Respiratory and Critical Care Medicine, Wuming Hospital of Guangxi Medical University, Nanning, Guangxi, 530199, People’s Republic of China; 3Radiological department, Wuming Hospital of Guangxi Medical University, Nanning, Guangxi, 530199, People’s Republic of China; 4Clinical Laboratory, Wuming Hospital of Guangxi Medical University, Nanning, Guangxi, 530199, People’s Republic of ChinaCorrespondence: Minchao Duan, Department of Respiratory and Critical Care Medicine, Wuming Hospital of Guangxi Medical University, Nanning, Guangxi, 530199, People’s Republic of China, Tel/Fax +86 15507711966, Email musicladyd@sina.comBackground: 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.Keywords: co-infection, Nocardia terpene, Pneumocystis jiroveci, anti-synthetase syndrome, anti-Ro-52 antibody, anti-Jo-1 antibody

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. [ABSTRACT FROM AUTHOR]

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.

Abstract A 65‐year‐old female was admitted with rapidly progressive respiratory failure requiring intubation and mechanical ventilation. She was considered to have an infective exacerbation of underlying interstitial lung disease (ILD). She improved on antibiotics, but the interstitial process progressed rapidly, and she could not be weaned. An antimyositis antibody panel yielded a strongly positive anti‐Jo‐1 and anti‐Ro 52. A diagnosis of antisynthetase syndrome (ASS) associated ILD, a very rare disease with high mortality, was made. She was managed with high‐dose corticosteroids and intravenous immunoglobulin therapy and was eventually liberated from mechanical ventilation. This case highlights the importance of considering ASS in an otherwise unexplained rapidly progressive ILD requiring mechanical ventilation.

A 65‐year‐old female was admitted with rapidly progressive respiratory failure requiring intubation and mechanical ventilation. She was considered to have an infective exacerbation of underlying interstitial lung disease (ILD). She improved on antibiotics, but the interstitial process progressed rapidly, and she could not be weaned. An antimyositis antibody panel yielded a strongly positive anti‐Jo‐1 and anti‐Ro 52. A diagnosis of antisynthetase syndrome (ASS) associated ILD, a very rare disease with high mortality, was made. She was managed with high‐dose corticosteroids and intravenous immunoglobulin therapy and was eventually liberated from mechanical ventilation. This case highlights the importance of considering ASS in an otherwise unexplained rapidly progressive ILD requiring mechanical ventilation. [ABSTRACT FROM AUTHOR]

Pulmonary hypertension (PH) is a serious condition in which there is an abnormally high pressure in the pulmonary arteries that can occur as a complication of connective tissue diseases. Although the relationship between PH and systemic lupus erythematosus or systemic sclerosis has been well-characterized, PH rarely occurs in patients with anti-synthetase syndrome (ASS), and little is known about the pathophysiology and clinical outcome of patients with ASS-PH. We herein report a patient with anti-Jo-1-positive ASS complicated by PH and discuss the treatment strategy through a review of previously reported cases.

Background: Antisynthetase syndrome (AS) is an uncommon and under-recognised connective tissue disease characterized by the presence of antibodies to anti-aminoacyl t-RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS. Materials and Methods: This was a 2-year (2013-2014) retrospective analysis of patients diagnosed with anti-Jo-1-related AS. The presence of anti-Jo-1 antibody was tested by the immunoblot assay. All patients underwent high-resolution computed tomography of the chest, transthoracic echocardiography and evaluation for inflammatory myositis. Transbronchial lung biopsies and muscle biopsies were obtained when clinically indicated. Results: Nine patients (mean age: 43.8 years) were diagnosed with anti-Jo-1-related AS. The median duration of symptoms before diagnosis of AS was 6 months. All patients were negative for antinuclear antibodies by indirect immunofluorescence. The prevalence of ILD, myositis and arthritis at presentation was 100%, 77.8% and 55.6%, respectively. The most common ILD pattern was non-specific interstitial pneumonia (n = 6) followed by organizing pneumonia (n = 2) and usual interstitial pneumonia (n = 1). ILD was the sole manifestation in two patients and was subclinical in two patients. Six patients had pleuropericardial effusions, three patients had pulmonary artery hypertension and two patients had venous thromboembolism. Eight of the nine patients improved after treatment with steroids and other immunosuppressants. Conclusion: Antisynthetase syndrome is an important and a treatable cause of ILD. Strong clinical suspicion is needed to achieve an early diagnosis.

Anti-Synthetase Syndrome (ASyS) is a rare chronic autoimmune disorder characterized by myositis, interstitial lung disease (ILD), polyarthralgia, “mechanic’s hands” and Raynaud’s phenomenon. Liver lesions are quite rare in ASyS. In our ASyS case, we will discuss a 58-year-old man presenting with muscle weakness, arthralgia, and interstitial lung disease (ILD). He was positive for anti-Jo-1 antibodies, substantiating the diagnosis, and was started on treatment. This was followed by the appearance of a liver mass that disappeared when the patient achieved remission.

Wong type dermatomyositis describes a small group of patients with dermatomyositis clinically presented with pityriasis rubra pilaris-like eruptions, including diffuse palmoplantar hyperkeratosis and follicular hyperkeratosis. Histopathologic findings include follicular hyperkeratosis and arrector pili myositis. There have been 20 cases reported since Wong's large series. We report a 56-year-old male with overlap syndrome of Wong type dermatomyositis and rheumatoid arthritis. He also fulfilled the criteria of anti-tRNA synthetase syndrome due to the presence of anti-Jo-1 antibody and clinical features of both rheumatoid arthritis and myositis. No internal malignancy was found after a complete systemic survey and within 1 year follow-up period. There was a good response to oral steroid therapy.

Background: Antisynthetase syndrome is a rare and debilitating multiorgan disease characterized by inflammatory myopathy, interstitial lung disease, cutaneous involvement, and frequent chronic inflammation of the joints. Standard treatments include corticosteroids and immunosuppressants. In some cases, treatment resistance may develop. Administration of immunoglobulins intravenously is recommended in patients with drug-resistant antisynthetase syndrome.Case Presentation: Here, we describe the case of a 56-year-old woman of Algerian origin. 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. The administration of immunoglobulin subcutaneously was introduced at a dose of 2 g/kg per month and was well tolerated. Clinical improvement was observed within 3 months of initiation of subcutaneous administration of immunoglobulin. After 22 months of treatment, she showed a significant improvement in terms of muscle strength, pulmonary involvement, arthralgia, and immunodeficiency. Her serum creatine phosphokinase and C-reactive protein levels remained normal. Finally, she was compliant and entirely satisfied with the treatment.Conclusions: 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. This treatment also allowed, in our case, for the regression of the chronic immunodeficiency secondary to rituximab treatment. [ABSTRACT FROM AUTHOR]

Background: Antisynthetase syndrome (AS) is an uncommon and under‑recognised connective tissue disease characterized by the presence of antibodies to anti‑aminoacyl t‑RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS. Materials and Methods: This was a 2‑year (2013‑2014) retrospective analysis of patients diagnosed with anti‑Jo‑1‑related AS. The presence of anti‑Jo‑1 antibody was tested by the immunoblot assay. All patients underwent high‑resolution computed tomography of the chest, transthoracic echocardiography and evaluation for inflammatory myositis. Transbronchial lung biopsies and muscle biopsies were obtained when clinically indicated. Results: Nine patients (mean age: 43.8 years) were diagnosed with anti‑Jo‑1‑related AS. The median duration of symptoms before diagnosis of AS was 6 months. All patients were negative for antinuclear antibodies by indirect immunofluorescence. The prevalence of ILD, myositis and arthritis at presentation was 100%, 77.8% and 55.6%, respectively. The most common ILD pattern was non‑specific interstitial pneumonia (n = 6) followed by organizing pneumonia (n = 2) and usual interstitial pneumonia (n = 1). ILD was the sole manifestation in two patients and was subclinical in two patients. Six patients had pleuropericardial effusions, three patients had pulmonary artery hypertension and two patients had venous thromboembolism. Eight of the nine patients improved after treatment with steroids and other immunosuppressants. Conclusion: Antisynthetase syndrome is an important and a treatable cause of ILD. Strong clinical suspicion is needed to achieve an early diagnosis. [ABSTRACT FROM AUTHOR]

Abstract: Wong type dermatomyositis describes a small group of patients with dermatomyositis clinically presented with pityriasis rubra pilaris-like eruptions, including diffuse palmoplantar hyperkeratosis and follicular hyperkeratosis. Histopathologic findings include follicular hyperkeratosis and arrector pili myositis. There have been 20 cases reported since Wong's large series. We report a 56-year-old male with overlap syndrome of Wong type dermatomyositis and rheumatoid arthritis. He also fulfilled the criteria of anti-tRNA synthetase syndrome due to the presence of anti-Jo-1 antibody and clinical features of both rheumatoid arthritis and myositis. No internal malignancy was found after a complete systemic survey and within 1 year follow-up period. There was a good response to oral steroid therapy. [Copyright &y& Elsevier]

Background: Anti-synthetase syndrome associated interstitial lung disease can occur either simultaneously, before, or after the development of polymyositis/dermatomyositis. Histology of interstitial lung disease can be nonspecific interstitial pneumonia, usual interstitial pneumonia, diffuse alveolar damage, organizing pneumonia. Organizing pneumonia associated anti-synthetase syndrome is a rare finding especially as the first manifestation. Case presentation: We report a 41 year old male patient who presented with organizing pneumonia and 2 years following the onset, developed polymyositis with anti-JO-1 antibody positivity. Conclusion: It is important to screen patients with organizing pneumonia for anti-synthetase syndrome which can be manifested later. [ABSTRACT FROM AUTHOR]

Objective: To investigate the clinical and immunological features of cardiac involvement in patients with dermatomyositis (DM). Methods: Data of 271 adult patients with DM diagnosed in the Department of Rheumatology and Immunology, Peking University People's Hospital from 2003 to 2018 were collected retrospectively and analyzed statistically. Results: The occurrence of cardiac involvement in DM was 15.9% (43/271). Main feature of cardiac involvement in DM was elevated cardiac troponin I (cTnI). The most common abnormalities of ECG were T wave abnormality (27.9%, 12/43), sinus tachycardia (16.3%,7/43), ST-T change (14%, 6/43) and right bundle branch block (7%, 3/43). The common manifestations of echocardiography were left ventricular diastolic dysfunction (23.3%, 10/43) and pericardial effusion (23.3%, 10/43). As compared with DM patients without cardiac involvement, DM patients with cardiac damage were more likely to have rapidly progressive interstitial lung disease (ILD), skin damage, anemia, elevated creatine kinase, decreased C3 and serum albumin ( P< 0.05). Positive anti-Ro-52 antibody and Jo-1 antibody were detected more common in DM with cardiac involvement( P< 0.05). Conclusions: Cardiac damage is common complication of DM. Manifestations of cardiac damaging are varied. Rapid progressive ILD and positive Jo-1 and Ro-52 antibodies are more common in this group. Clinicians should improve the awareness of cardiac involvement in DM patients.

Background: Anti-synthetase syndrome associated interstitial lung disease can occur either simultaneously, before, or after the development of polymyositis/dermatomyositis. Histology of interstitial lung disease can be nonspecific interstitial pneumonia, usual interstitial pneumonia, diffuse alveolar damage, organizing pneumonia. Organizing pneumonia associated anti-synthetase syndrome is a rare finding especially as the first manifestation., Case Presentation: We report a 41 year old male patient who presented with organizing pneumonia and 2 years following the onset, developed polymyositis with anti-JO-1 antibody positivity., Conclusion: It is important to screen patients with organizing pneumonia for anti-synthetase syndrome which can be manifested later.