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)

A 34-year-old woman with stable systemic lupus erythematosus (SLE) treated with low-dose prednisone and hydroxychloroquine developed multiple bilateral pulmonary nodules. Open lung biopsy documented lymphocytic interstitial pneumonitis (LIP). LIP should be considered in the differential diagnosis of nodular pulmonary lesions in patients with SLE.
Lupus 1995 Apr
PMID:Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus. 779 24

The presence of antiphospholipid (aPL) antibodies and antiphospholipid syndrome (APS) was researched in 57 children and adolescents with systemic lupus erythematosus (SLE). The frequency of aPL antibodies was 75.4% (anticardiolipin 70.2% and lupus anticoagulant 29.1%). The positivity for these antibodies fluctuated during the course of the disease. No association was found between aPL antibodies and clinical or laboratory manifestations or the autoantibodies studied, nor with the activity or gravity of the SLE. APS was diagnosed in 14% of the cases (eight patients), on average three years after the diagnosis of SLE. Four patients had arterial thrombosis (stroke, three; transient ischaemic attack, one; amaurosis fugax, two; renal, one), one presented with deep vein thrombosis (DVT) and three had involvement of small calibre vessels (osteonecrosis, two; transverse myelitis, one). Recurrences were observed in three of the eight cases (37.5%), with a mean interval of 13 months between the events. The presence of APS was associated with haemolytic anaemia, leukopenia, thrombocytopenia, coagulation abnormalities, ischaemic cerebrovascular accidents, amaurosis fugax, osteonecrosis and interstitial pneumonitis. A negative association was observed between APS and the presence of anti-Ro antibodies.
Lupus 2003
PMID:Antiphospholipid antibodies and antiphospholipid syndrome in 57 children and adolescents with systemic lupus erythematosus. 1466 97

The objective of this study was to assess the prevalence, clinical, histological and immunological characteristics, and the long-term outcome of polymyositis- (PM) and dermatomyositis- (DM) associated lung disease, and to define subgroups of lung-associated inflammatory myopathies. This retrospective study included 81 consecutive patients diagnosed with PM/DM. Pulmonary involvement was systematically investigated in relation to clinical symptoms by chest radiography, high resolution computed tomography and pulmonary function testing. Anti-synthetase autoantibodies (ASA) were analysed by ELISA and confirmed by protein and RNA immunoprecipitation methods. Statistical analyses were done with the Student t-test and Fisher exact test. Cumulative survival probabilities were estimated by the Kaplan-Meier method and Cox regression analysis. Fifty patients (61%) presented pulmonary involvement. Thirty-two (39%) had interstitial lung disease and five of them had devastating acute interstitial pneumonia with pneumomediastinum and an unfavorable prognosis. Histology showed diffuse alveolar damage in this subgroup and ASA were negative. Eighteen patients (22%) presented restrictive myopathic lung disease; in three of them respiratory muscles could not maintain ventilation. ASA were positive in 17 of the 50 patients (34%) and were significantly associated with interstitial lung disease (OR: 4.5 [95% CI: 1.3-15.3]), arthritis (OR: 6.0 [95% CI: 1.3-29.2]) and 'mechanic hands' (OR: 8.5 [95% CI: 1.7-41.4]); the presence of these autoantibodies did not imply worse survival prognosis. We concluded that clinical and immunological characteristics allowed the grouping of patients into different types of PM/DM lung-associated disease. Presence of ASA did not affect survival. ASA-negative patients with acute interstitial pneumonitis and pneumomediastinum had an unfavorable prognosis.
Lupus 2005
PMID:Polymyositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients. 1721

Interstitial lung disease (ILD) in patients with polymyositis (PM) and dermatomyositis (DM) is often resistant to treatment and life threatening, being recognized as one of the severest complication in these autoimmune disorders. Patients with clinically amyopathic dermatomyositis (CADM) or those with anti-CADM140/MDA5 antibody are especially prone to develop rapidly progressive interstitial pneumonia. We retrospectively analyzed 46 patients with PM/DM admitted to our hospital and identified DM, rapidly progressive disease, honeycomb lung, CADM and extensive ILD as risk factors for recurrence or death. In the presence of two or more risk factors, the sensitivity and specificity for the prediction of death or relapse were 81.3% and 76.7%, respectively. Calcineurin inhibitors have been widely used as induction and maintenance therapy for PM/DM-associated ILD. Recently we reported the benefit of tacrolimus on the disease-free survival and event-free survival of the patients with PM/DM-associated ILD. Among those patients treated with tacrolimus, poor prognostic factors for death, recurrence or severe adverse event were identified as acute progression of the disease, honeycomb lung, forced vital capacity (FVC) less than 80% and having DM. The potential effectiveness of an intensive therapy protocol with triple therapy that comprises high-dose corticosteroids, calcineurin inhibitors and cyclophosphamide has been reported.
Lupus 2015 Jan
PMID:The efficacy of calcineurin inhibitors for the treatment of interstitial lung disease associated with polymyositis/dermatomyositis. 2529 51