Prognostic value of myositis-specific antibodies in patients with idiopathic interstitial pneumonia.

BACKGROUND: Patients with idiopathic interstitial pneumonia (IIP) often exhibit positivity for myositis-specific antibodies (MSA). However, the significance of this finding remains unclear. In this study, we investigated the association of MSA with the prognosis and risk of acute exacerbation in patients with IIP. METHODS: We retrospectively reviewed the medical records of patients with IIP and examined the effect of each MSA subtype on survival and acute exacerbation. RESULTS: Of 240 patients with IIP, 48 (20%) exhibited positivity for MSA. The MSA subtypes included: PL-7 (antithreonyl; n = 16, 6.7%); signal recognition particle (n = 13, 5.4%); PL-12 (antialanyl; n = 9, 3.8%); Mi-2 (n = 8, 3.3%); OJ (anti-isoleucyl; n = 7, 2.9%). During the 382 days (382 ± 281 days) of observation, 32 (13%) patients expired, and 27 (11%) experienced an acute exacerbation. Cox proportional hazards regression analysis demonstrated that age at the initial visit (hazard ratio [HR]: 1.072; 95% confidence interval [CI]: 1.017-1.131; P = 0.01), PL-7 (HR: 4.785; 95% CI: 1.528-14.925; P = 0.007), and PL-12 (HR: 3.922; 95% CI: 1.198-12.82; P = 0.024) were independent predictors of survival time. PL-7 (HR: 3.268; 95% CI: 1.064-10; P = 0.039) and PL-12 (HR: 5.747; 95% CI: 1.894-7.544; P = 0.002) were independent predictors of time from first visit to acute exacerbation. CONCLUSION: Detecting MSA in patients with interstitial lung disease may be useful in predicting prognosis and providing a rationale for intensive treatment.

Anti-PL-12 anti-synthetase syndrome manifesting with multiple digital ischemia: Case report & review of the literature.

Key Clinical Message: Acute digital ischemia is a rare manifestation of anti-synthetase syndrome in the absence of Raynaud's phenomenon. A high index of suspicion may result in early diagnosis and better clinical outcomes. Abstract: A 61-year-old male patient was admitted to the hospital for worsening arthralgias with morning stiffness lasting hours, as well as left sided headaches, and jaw pain while eating. He had significant weight loss and subjective fever at home. Multiple fingers and toes were noted to be ischemic. His laboratory workup was pertinent for significantly elevated inflammatory markers, and mild Creatinine kinase elevation. Chest imaging and later lung biopsy were notable for organizing pneumonia. Conventional angiogram showed evidence of significant digital disease without collaterals. Subsequent autoimmune screening tests with extended myositis-specific and myositis-associated panels revealed a strongly positive anti-PL-12 antibody and moderately positive anti- SSA-52KD IgG ab. After ruling out infectious etiologies and malignancy, anti-synthetase syndrome (ASyS) diagnosis was considered in the presence of ischemic digits, organizing pneumonia, polyarthralgia, constitutional symptoms, increased inflammatory markers and positive antibodies. The patient was treated with high dose prednisone and mycophenolate mofetil along with amlodipine and sildenafil for digital vasodilation. Acute digital ischemia may be the first manifestation of ASyS with ILD. A high index of suspicion is warranted for early diagnosis and better outcomes.

IVIG for refractory dysphagia in Antisynthetase syndrome: A truth hard to swallow.

Refractory dysphasia could be the main symptom of Antisynthetase syndrome (ASS). IVIG may have a major impact in the successful treatment of dysphasia in patients with ASS. In our patient with ASS, IVIG treatment was an unreplaceable treatment option, and the patient regains her ability to swallow within 2 days.

Dermatomyositis Which Was Double Positive for Anti-MDA5 and Anti-ARS Antibodies That Was Successfully Treated by Intensive Immunosuppressive Therapy.

Myositis-specific autoantibody is associated with the clinical phenotype and prognosis of dermatomyositis. Anti-melanoma differentiation-associated gene 5 (MDA5) and anti-aminoacyl-tRNA synthetase (ARS) antibodies are generally mutually exclusive. We herein present an extremely rare case of dermatomyositis which showed double positivity for anti-MDA5 and anti-ARS antibodies. There have been very few reported cases of double positive anti-MDA5, anti-ARS antibodies. In such cases, the clinical characteristics of each autoantibody can coexist. Thus, we should pay attention to the rapidly progressing features of anti-MDA5 as well as the chronic relapsing features of anti-ARS for the better management of this rare condition.

Anti-Synthetase Syndrome-Related Interstitial Lung Disease With Anti-PL-12 Antibodies.

Anti-synthetase syndrome usually comprises interstitial lung disease, myositis, arthralgias, and Raynaud phenomenon. The anti-PL-12 antibody is directed against the enzyme alanyl-tRNA synthetase and has been associated with interstitial lung disease in the absence of inflammatory myositis. We report the case of a 33-year-old woman with complaints of progressive dyspnea, a persistent dry cough, along with intermittent low-grade fever for a few months. A computed tomography (CT) scan of the chest showed the presence of patchy bilateral airspace opacities and infiltrates. It also showed significant mediastinal and hilar lymphadenopathy. Bronchoscopy with transbronchial biopsy was performed, and histopathology changes were consistent with connective tissue disease related to interstitial lung disease. Further workup revealed the presence of anti-PL-12 antibodies. This case illustrates a rare association of interstitial lung disease with the anti-PL-12 antibody.

Dermatomyositis with Rapidly Progressive Interstitial Lung Disease Treated with Rituximab: A Report of 3 Cases in Japan.

We performed a retrospective chart review of three patients with hypomyopathic dermatomyositis and rapidly progressive interstitial lung disease. The patients were Japanese women of 71, 69, and 65 years of age. Two patients were anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and 1 was anti-aminoacyl-tRNA synthetase (anti-ARS) antibody-positive. Their respiratory statuses deteriorated despite the administration of glucocorticoid, calcineurin inhibitors, and intravenous cyclophosphamide therapy. We subsequently administered rituximab. The anti-ARS antibody-positive patient survived, while 2 anti-MDA5 antibody-positive patients died.

Anti-synthetase syndrome associated with anti PL-12 and anti-Signal recognition particle antibodies and a necrotizing auto-immune myositis.

We report a 37-year-old woman with a 2 month history of proximal muscle weakness and extremely high creatine kinase (21,808 U/L) due to necrotizing auto-immune myositis (NAM) in association with anti-synthetase syndrome. Myositis-specific auto-immune antibody panel was positive for anti-Signal recognition particle and anti-PL-12. CT scan of the chest confirmed interstitial lung disease. Prednisolone, intravenous immunoglobulin and cyclophosphamide therapy was given with gradual improvement. This patient is notable for the unusual combination of NAM and anti-synthetase syndrome with the rare finding of two myositis-specific autoantibodies, which directed testing for associated extramuscular features and management with more aggressive immunotherapy.