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.

We experienced a rare case of severe contractured and flexed metacarpophalangeal joint (MPJ) arthropathy in a patient with overlap syndrome of systemic lupus erythematosus and dermatomyositis. The patient was a 60-year-old female with high anti-nuclear antibody and anti-Jo-1 antibody titers whose hands had gradually deformed without arthralgia during the past 6 years. Both hands were extremely contractured; in particular, the right hand had dislocations of the second to fifth MPJs, ankyloses of some proximal interphalangeal and distal interphalangeal joints, and z-shaped deformity of the thumb. We performed thumb arthroplasty and artificial joint replacement of the second to fifth MPJs of the dominant right hand. These operations enabled improvement of the patient's ability to perform daily activities, with an improvement from 1.125 to 0.625 of the Japanese version of the Stanford Health Assessment Questionnaire score. Although surgery to treat contractured deformities can only partially recover the functional ability, it also has indications for arthroplasties. [ABSTRACT FROM AUTHOR]

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]

OBJECTIVE: Investigating the correlation between children's status asthmatics and interstitial lung disease (ILD). PATIENTS AND METHODS: We continuously selected 20 cases of children's status asthmatics combined with ILD (group A), 20 cases of pure status asthmatics (group B), 20 cases of pure ILD (group C) and 20 cases of healthy children (group D). We measured Th1/Th2 by flow cytometry as well as the level of expression of hs-CRP, IL-7 cytokines (TSLP), monocyte chemoattractant protein-1 (MCP-1) and anti-Jo-1 antibody by ELISA method. RESULTS: Th1 and Th1/Th2 of groups A and B were significantly lower than those of group C and D. Th2 was significantly (p<0.05) higher than that of groups C and D. The level of expression of hs-CRP, TSLP, MCP-1 and anti-Jo-1 antibody in the groups A and B were all significantly higher (p<0.05) than those of groups C and D. There were differences of the above index of the comparison between groups A and B, but no difference between groups C and D. CONCLUSIONS: Children's status asthmatics and ILD may correlate with the abnormal expression of Th1/Th2, hs-CRP, TSLP, MCP-1 and anti- Jo-1 antibody. [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]

A small proportion of patients with rheumatoid arthritis (RA) develop idiopathic inflammatory myopathies (IIM); however, the clinical and immunological characteristics of these patients have not been elucidated. In the present study, we evaluate the frequency of autoantibodies and the accompanying clinical features in patients with IIM overlapped to RA (IIM-RA) and in patients with IIM without RA. Twelve patients with IIM-RA were selected from 142 patients with IIM who were admitted to our hospital. Clinical and laboratory data, including autoantibody test results, were collected from patient medical records. Myositis-specific antibodies (MSAs) were analyzed by immunoprecipitation. Clinically, patients with IIM-RA were more likely to be male, to have polymyositis, and to be older at the time of IIM onset than patients with IIM without RA. Patients with IIM-RA had been treated for 2-25 years prior to the onset of IIM with more than two disease-modifying antirheumatic drugs (DMARDs). Patients with IIM-RA had a high frequency (75.0%) of positivity for MSAs, including anti-Jo-1, anti-PL-7, anti-PL-12, or anti-signal recognition particle (SRP) antibodies; anti-Jo-1 antibody was detected in 4 patients (33.3%). In addition, 2 out of 12 patients with IIM-RA were concurrently positive for two different MSAs, anti-Jo-1, and anti-PL-7 antibodies. In 3 other patients with IIM-RA, anti-Jo-1 antibody, or anti-PL-7 antibody was detected in serum samples collected 6-18 months prior to development of myositis. High frequency and coexistence of MSAs were detected in patients with IIM-RA. MSAs detected in patients with RA even without symptoms of myositis may indicate possible future development of myositis. [ABSTRACT FROM AUTHOR]

Rheumatoid arthritis (RA) and antisynthetase syndrome (ASS) are distinct clinical syndromes, and their co-occurrence is rarely encountered. The authors report the case of a 56-year-old female patient with RA of 3 years duration who suddenly developed ASS, and include a review of the literature. The patient was diagnosed with ASS based on; positivity for anti-histidyl-tRNA synthetase (Jo-1) antibody, interstitial lung disease, polyarthritis, and mechanic's hands. High-dose corticosteroid and pulse intravenous cyclophosphamide were used to control the ASS. This case demonstrates that ASS should be considered during clinical presentations due to its potential overlap with RA. [ABSTRACT FROM AUTHOR]

Antitumor necrosis factor (TNF) therapy has been associated with adverse immunologic events including systemic lupus erythematosus. However, the development of polymyositis (PM)/dermatomyositis (DM) associated with anti-TNF therapy is extremely rare. We experienced a case of a 48-year-old female with rheumatoid arthritis (RA) who had anti-Jo-1 antibodies and interstitial lung disease but no previous history of PM/DM and who developed PM soon after the initiation of etanercept (ETN) therapy for RA. The patient recovered upon withdrawal from ETN and corticosteroid (CS) therapies. Only four reports of PM/DM associated with anti-TNF therapy for RA could be found in the literature. The patients described in three of the four reports were positive for anti-Jo-1 antibodies before the initiation of anti-TNF therapy, and in all the cases, recovery occurred after the cessation of anti-TNF-agent administration and CS therapy. These results suggest a relationship between the onset of PM/DM with anti-Jo-1 antibody and anti-TNF therapy for RA. [ABSTRACT FROM AUTHOR]

Abstract: Microparticles are recently recognized players of intercellular communication. They are involved in signal transduction, cell activation and apoptosis. Their importance is also suggested in autoimmune diseases such as rheumatoid arthritis and systemic sclerosis. We investigated the role of microparticles in polymyositis/dermatomyositis, a group of rare autoimmune diseases, characterized by specific skin lesions and muscle weakness. The plasma concentration of monocyte and lymphocyte derived microparticles of 20 patients with polymyositis/dermatomyositis and 20 healthy controls were determined by flow cytometry. The structure of microparticles was visualized by electron microscopy. Significantly elevated numbers of monocyte (CD14 positive), T-lymphocyte (CD3 positive) and B-lymphocyte (CD19 positive) derived microparticles were found in the plasma samples of polymyositis/dermatomyositis patients, compared to healthy controls (p =0.001, 0.01 and 0.006, respectively). Furthermore, the plasma levels of monocyte and B-lymphocyte derived microparticles correlated with the manual muscle strength test (r =0.497, p =0.027; r =0.508, p =0.023; respectively). Patients with anti-Jo-1 antibody and lung involvement had significantly higher numbers of T- and B-lymphocyte and monocyte derived MPs (p =0.006, 0.012 and 0.007, respectively, for anti-Jo-1; p =0.013, 0.016 and 0.025, respectively, for lung involvement). After ultracentrifugation, CK activity could be detected only in traces in the resuspended pellet containing microparticles of healthy and diseased individuals. The electron microscopy revealed slightly different microparticles in the samples of patients with polymyositis/dermatomyositis. These results suggest that immune cell derived microparticles may contribute to the inflammatory process in polymyositis/dermatomyositis, however, CK-positive, possibly muscle derived microparticles do not seem to be present in the blood of patients with polymyositis/dermatomyositis. [Copyright &y& Elsevier]

The article presents a case study of a 16-year-old Japanese boy with complaints of skin ulcer but no muscle weakness. He suffered from hepatic insufficiency at the age of 15 where abdominal ultrasound revealed hyperechogeneic liver. Physical examination revealed a butterfly rash on his face, several ulcers on his upper back, chest, and right elbow. Amyopathic dermatomyositis (ADM) complicated by acute interstitial pneumonia was diagnosed.

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]

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. The case is reported of a patient who suffered from shortness of breath and dry cough, with pulmonary fibrosis on CXR and CT scan, and interstitial pneumonitis on trans-bronchial biopsy. She was also positive for anti-Jo-1 and anti-Ro antibodies. ‘Mechanic’s hands' were noted bilaterally but with no evidence of myopathy in either the electromyogram or on muscle biopsy. The patient was treated with prednisolone and her clinical picture, including the ‘mechanic’s hands' and lung fibrosis, subsided gradually, suggesting that the sign may be a useful follow up tool in this disease. Early diagnosis and corticosteroid therapy could be beneficial for these patients. [ABSTRACT FROM AUTHOR]

Aim: To review the clinical features of dermatomyositis (DM) in a South Australian population. Methods: Retrospective review of medical records of patients with biopsy-proven DM in South Australia from 1990 to 2005. Results: There were 21 cases of biopsy-proven DM in SA (62% F, mean age 49.7 ± 18.4 years) and clinical details were available in 20 of these. Malignancy was identified in 9/20 patients; in five this followed the diagnosis of DM, with three malignancies seen within 3 months of disease onset. Three patients had a clearly defined immune insult prior to the diagnosis of DM; one patient had Mycoplasma pneumoniae infection 23 days prior to DM, two had pneumococcal and influenza vaccinations 5 and 14 days prior to the onset of DM, respectively. Two of three patients with anti-Jo-1 antibody experienced thromboembolism within 2 months of DM onset and three patients had interstitial lung disease (2 with anti-Jo-1 antibody). Creatine kinase (CK) was elevated in 15/20 cases and showed strong correlation with transaminases, and notably not with traditional inflammatory markers. Conclusions: This retrospective review of patients with biopsy-proven DM suggests a role for infection/vaccination in triggering disease onset. A particularly strong association with malignancy was observed and it is suggested that DM may predispose to thrombosis. Transaminases, in addition to CK may be used to monitor disease activity, and traditional inflammatory markers have little role in this. [ABSTRACT FROM AUTHOR]

Idiopathic inflammatory myopathies (IM), including dermatomyositis (DM) and polymyositis (PM), are a group of systemic rheumatologic diseases of unknown etiology characterized by chronic myositis. Antisynthetase antibodies such as the anti-Jo-1 antibody are known to be highly specific for inflammatory myopathies. Patients with this antibody frequently show a combination of symptoms including interstitial lung disease, fever, polyarthritis, myositis, Raynaud's phenomenon and "mechanic's hands". In the management of PM with anti-Jo-1 antibody, immunosuppressive agents are used to control the disease. Leflunomide is a new immunosuppressive drug recently introduced in the treatment of rheumatoid and psoriatic arthritis. Here, we report two cases of female patients with PM and anti-Jo-1 antibodies, who were successfully treated with leflunomide. [ABSTRACT FROM AUTHOR]

Anti-Jo-1 antibody is a myositis specific autoantibody most commonly found in patients with idiopathic inflammatory myopathies (IIM). This antibody is directed against the histidyl-tRNA synthetase which catalyses the binding of the histidine to its cognate tRNA during protein synthesis. It can be considered a specific marker of IIM, predominantly found in 20–30% of patients with PM and in the 60–70% of those with interstitial pulmonary fibrosis. These antibodies are also found in DM, although less frequently than in PM, and are rare in children with PM or DM and in other connective tissue diseases.ELISA, CIE and immunoblotting are highly specific and sensitive techniques for testing anti-Jo-1 antibodies. The detection of this antibody is particularly useful in diagnosis and classification of IIM. Moreover, anti-Jo-1 serum levels strongly correlate with disease activity representing a good marker for disease monitoring. [ABSTRACT FROM AUTHOR]

Introduction: Antisynthetase Syndrome is associated with interstitial lung disease in adult patients, but this has not been described in children. Materials and methods: 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. Conclusion: We present the clinical, radiographic, and histological findings of a child with interstitial lung disease due to Antisynthetase Syndrome. [ABSTRACT FROM AUTHOR]

• NMOSD with complete spinal cord involvement is rare and overlooked. • Clinical manifestations of NMOSD and ASS overlap each other. • NMOSD must be considered in the diagnosis of patients with ASS with myelopathy. • Therapeutics targeting B cells are effective in NMOSD coexisting with ASS. Anti-synthetase syndrome (ASS) is a rare autoimmune disorder characterized by the presence of antibodies against aminoacyl-transfer RNA synthetase commonly associated inflammatory myopathy. In this case report, we describe an adult female with NMOSD concurrent with ASS in which the lesion involved the entire length of the spinal cord. Since B-cell mediated molecular pathway is involved in the pathogenesis of NMOSD and ASS, we suggest that the therapeutically targeted killing of B-cells, such as Rituximab, is effective. [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.

A 57-yr-old lady developed polymyositis whilst taking D-penicillamine for RA. D-Penicillamine-induced polymyositis occurs in RA with a greater frequency than idiopathic polymyositis. Anti-acetyl choline receptor antibodies and ANA were positive, consistent with drug-induced disease. Anti-Jo-1 antibodies are considered specific for idiopathic myositis, and their presence was unexpected. Following withdrawal of the drug, the disappearance of the anti-Jo-1 and other antibodies coincident with clinical improvement, suggested that D-penicillamine was responsible for inducing antibody production. [ABSTRACT FROM PUBLISHER]

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.