Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0206061 (interstitial pneumonia)
 6,105 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present two cases of interstitial pneumonia (IP) whose sera contain autoantibodies to PL-12 (alanyl tRNA synthetase). The first patient is a 47-year-old female who was diagnosed as IP and treated with corticosteroid at another hospital. She was admitted to Keio University Hospital due to worsening of dyspnea and polyarthritis. Laboratory studies revealed elevation of LDH and CRP, and her chest radiography showed interstitial fibrosis. Because of clinical deterioration, the dose of corticosteroid was increased (prednisolone 40 mg/day) and her symptom was stabilized. The second patient, a 55 year-old female, was admitted to Tokyo Metropolitan Ohtsuka Hospital because of dyspnea on exertion and polyarthritis. She did not show any symptom of myositis and was diagnosed as IP with arthritis on the basis of her clinical and chest radiography. She was treated with oral corticosteroid (prednisolone 30 mg/day), which resulted in improvement of her respiratory symptom and arthritis. Both patients were found to have autoantibodies to the PL-12. Autoantibodies to aminoacyl tRNA synthetases have been recognized as having a linkage with myositis mainly because of observations of the Jo-1 specificity. There was one report on a North American population that most but not all patients with anti-PL-12 antibodies had myositis. However, the clinical significance of anti-PL-12 has not been examined in Japanese patients. These patients suggested that anti-PL-12 antibodies have a stronger association with IP than myositis in Japanese patients.
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PMID:[Two cases of interstitial pneumonia with anti-PL-12 (alanyl tRNA synthetase) antibodies]. 912 26

We present two cases of polymyositis (PM) associated with interstitial pneumonia (IP) whose sera contain autoantibodies to OJ (isoleucyl tRNA synthetase). The first patient is a 51 year-old female who was diagnosed as rheumatoid arthritis (RA) and treated with gold and corticosteroid at another hospital. She was admitted to Keio University Hospital due to worsening of dyspnea on exertion and polyarthritis. Laboratory findings revealed elevation of serum CK and LDH. A diagnosis of PM was made based on the myogenic pattern of EMG and pathological feature by muscle biopsy. Chest radiography and CT showed interstitial fibrosis. Because of clinical deterioration, the dose of corticosteroid was increased (prednisolone 50 mg/day) and her symptom was stabilized. The second patient, a 62 year-old male, was admitted to Kawasaki Municipal Hospital because of dyspnea on exertion, polyarthritis, and fever. He was diagnosed as PM associated with IP on the basis of his clinical and laboratory findings, and chest radiography. He was treated with methylprednisolone pulse therapy (800 mg/day for three days) and his symptoms were improved. Both patients were found to have autoantibodies to OJ. Autoantibodies to aminoacyl tRNA synthetase have been described to be associated with myositis and/or IP. In North American, it was reported that all patients with anti-OJ had either myositis or IP or both. This suggests that anti-OJ was commonly associated with the anti-synthetase syndrome observed with other anti-synthetases. This is the first report of Japanese patients with anti-OJ antibody. The clinical features of these patients were likely to be similar to those observed in North American patients. However, further studies are necessary to clarify the precise clinical significance of this antibody.
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PMID:[Two cases of polymyositis associated with interstitial pneumonia with anti-OJ (isoleucyl tRNA synthetase) antibodies]. 972 63

Pulmonary involvement is a common feature in patients with various collagen diseases. Some types of the pulmonary involvement are resistant to currently available treatment regimens and thus considered as intractable conditions. These include acute/subacute interstitial pneumonia in dermatomyositis, pulmonary interstitial fibrosis in scleroderma, and diffuse alveolar hemorrhage. Acute/subacute interstitial pneumonia with the histology of diffuse alveolar damage (DAD) is mainly occurred in patients with amyopathic or hypomyopathic dermatomyositis who lack autoantibodies to aminoacyl tRNA synthetases. Intensive immunosuppressive treatment in the early phase of the disease may be effective for this intractable complication. Nearly one-third of patients with scleroderma have slowly progressive pulmonary interstitial fibrosis, leading to end-stage respiratory failure. Non-specific interstitial pneumonia (NSIP) with an excessive fibrotic change is a major histology of these patients. There are accumulating evidences showing the effectiveness of cyclophosphamide in patients with this intractable condition, especially those with active alveolitis. Diffuse alveolar hemorrhage is a fatal complication mainly occurred in patients with systemic lupus erythematosus and those with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, including microscopic polyangitis (MPA) and Wegener's granulomatosus. Immediate diagnosis and introduction of intensive treatment are necessary to save the patients with this complication.
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PMID:[Collagen diseases with pulmonary involvement]. 1529 Dec 48

Autoantibodies against aminoacyl-tRNA synthetase (ARS) are known to associate with myositis, arthritis, and interstitial lung disease (ILD). Anti Jo-1 antibody is a common diagnostic tool of this syndrome and is available on a commercial basis, while other anti-ARS antibodies such as anti-EJ, anti-PL7 and anti-OJ antibodies are less commonly examined. The aim of this study was to investigate the clinicopathological characteristics of 9 ILD patients who were positive for anti-ARS antibodies including anti-EJ, anti-PL7 and anti-Jo-1 antibodies. In physical examination, muscle pain and/or skin rash (including mechanic's hand) were observed frequently. In laboratory findings, levels of myogenic enzymes were slightly elevated, and anti-nucleus antibody was detected in most cases. Air-space consolidation with traction bronchiectasis, ground glass opacities and characteristic volume loss of both lower lobes were observed in radiological examination. Organization in alveolar spaces and lymphoid alveolitis that did not destroy existing pulmonary structures were main pathological findings. Immunosupressive therapy was effective and the prognosis appears satisfactory in these cases. Although anti-ARS antibodies had been measured as a marker of myositis, we suggest that they are useful as markers of interstitial pneumonia.
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PMID:[Clinical and pathological findings of patients with interstitial lung disease associated with antisynthetase]. 1636 63

Polymyositis and interstitial lung diseases, predominantly nonspecific interstitial pneumonia (NSIP), are known to be frequent in antisynthetase syndrome, where anti-aminoacyl-tRNA synthetase antibodies are often identified. An unusual case of acute respiratory distress syndrome, secondary to such proven NSIP of cellular type with predominant CD8 lymphocytes, is described herein. The patient described in the present case study initially had a poor recovery with high dose of steroids, but this was followed by a good improvement after the prescription of tacrolimus and a low dose of prednisone. A precise diagnosis in similar circumstances may be life-saving, allowing the successful application of new immunosuppressants.
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PMID:Acute respiratory distress syndrome secondary to antisynthetase syndrome is reversible with tacrolimus. 1859 48

A 51-year old man with severe acute respiratory failure was referred to our hospital. He presented with cyanotic digits, high fever and dyspnea. The chest radiograph and chest CT scan showed diffuse ground-glass-opacities and reticular shadows in both lung fields. We diagnosed acute interstitial pneumonia. The pulmonary infiltration seen on chest radiograph diminished gradually after pulse methylprednisolone and cyclophosphamide therapy. Later, we screened his sera for antibodies to anti-aminoacyl tRNA synthetase to diagnose anti-aminoacyl tRNA synthetase syndrome because anti-PL-12 antibodies were positive. This case has been followed but no myositis or recurrence of interstitial pneumonia has been recognized.
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PMID:[A case of rapidly progressive interstitial pneumonia with anti-pL-12-antibodies successfully treated by pulse methylprednisolone and cyclophosphamide]. 1870 May 73

Past paradigm that the muscle tissue injury is driven by CD4 T cells and associated humoral immunity in dermatomyositis (DM), and by cytotoxic T cells in polymyositis (PM) is now under challenge. Although pathogenic autoantigens are to be identified, skeletal muscle C-protein was an excellent immunogen to provoke experimental myositis mimicking human PM. Serum antibodies against aminoacyl tRNA synthases appear in PM/DM patients, but more often in interstitial pneumonitis patients. Inflammatory cytokines are obviously involved in the pathogenesis. Animal studies showed that autoimmune myositis occurs without tumor necrosis factor alpha. Indeed, its blockade has yielded inconsistent outcome. Most crucial ones will be therapeutic targets in the future.
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PMID:[Recent advance in polymyositis and dermatomyositis research]. 1928 Sep 27

We report two cases of biopsy-proven nonspecific interstitial pneumonia (NSIP) with anti-KS (asparaginyl-tRNA) antibody. Anti-KS antibody is the sixth anti-aminoacyl-tRNA synthetase (ARS) antibody. They showed interstitial pneumonia without clinical symptoms, and high resolution computed tomography (HRCT) of the chest demonstrated consolidation along bronchovascular bundles and volume loss in the bilateral lower lobes, which were suggestive of connective tissue diseases (CTD). One case of cellular NSIP responded to corticosteroid, but the other case of fibrotic NSIP required corticosteroid and cyclosporin. In patients with these HRCT findings, the measurement of anti-ARS antibodies could be helpful even in the absence of clinical symptoms suggestive of CTD.
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PMID:Nonspecific interstitial pneumonia (NSIP) associated with anti-KS antibody: differentiation from idiopathic NSIP. 1965 35

A 54-year-old woman, complaining of a dry cough in the beginning of December 2004, was admitted to our hospital because of its exacerbation in January 2005. Interstitial pneumonia was suspected according to a chest Xray and chest CT. The pathological diagnosis of cellular nonspecific interstitial pneumonia (NSIP) was made after video assisted thoracic surgery. She did not have muscle weakness or arthralgia but she had a skin lesion ("mechanic's hand") which is often seen in patients with antisynthetase syndrome. Anti-Jo-1 antibody was negative but anti-OJ antibody was positive. Therefore, anti-aminoacyl tRNA synthetase syndrome with cellular NSIP was diagnosed. She had a favorable response to the initial treatment of methylprednisolone pulse therapy followed by prednisolone 1 mg/kg/day. Her symptoms, pulmonary function test and chest imaging findings have showed improvement after therapy.
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PMID:[A case of cellular NSIP with anti-OJ (anti-isoleucyl tRNA synthetase) antibodies]. 2016 21

Of the idiopathic inflammatory myopathies, the anti-aminoacyl tRNA synthetase syndrome has the greatest association with interstitial lung disease (ILD). We reviewed 13 open surgical lung biopsies, four autopsies, and three native lungs resected at transplantation, for pulmonary ILD associated with the presence of anti-histidyl tRNA synthetase (anti-Jo-1) autoantibodies. Fifty percent (N=10) of patients presented with an acute decompensation of pulmonary function manifested as diffuse alveolar damage, although in five patients (25%) this marked diminution in function was superimposed on an underlying chronic interstitial pneumonia (usual interstitial pneumonia (three); nonspecific interstitial pneumonia(two)). Seven (35%) patients had usual interstitial pneumonia and two (10%) had nonspecific interstitial pneumonia exclusively, whereas one patient presented with an organizing pneumonia (5%). This study is the first to highlight the high biopsy incidence of diffuse alveolar damage in this patient population both de novo and superimposed on underlying chronic ILD, and also shows that usual interstitial pneumonia remains a significant pattern of interstitial injury in this autoimmune group. On the basis of coexisting patterns of lung injury, this study also suggests that nonspecific interstitial pneumonia in connective tissue disorders may progress over time to a usual interstitial pneumonia pattern of fibrosis, an observation that could be better assessed with future inclusion of autopsy and transplanted native lungs in study groups.
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PMID:The pulmonary histopathologic manifestations of the anti-Jo-1 tRNA synthetase syndrome. 2022 83


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