bromochloroacetic-acid and Dermatomyositis

Pulmonary manifestations contribute significantly to the morbidity and mortality of the idiopathic inflammatory myopathies, ranging from intrinsic lung disease to secondary complications that include aspiration pneumonia, opportunistic infection, congestive heart failure, and hypoventilation. Newer classification schemes for interstitial lung disease have permitted closer correlation between histologic subtype and clinical outcome, while diagnostic techniques such as bronchoalveolar lavage have begun to define the cellular elements responsible for immune-mediated pulmonary dysfunction. Investigators have identified several serum markers correlating with inflammatory disease activity in the lung that should enhance noninvasive monitoring of therapeutic responses to newer regimens involving agents such as cyclosporine and tacrolimus. Taken together, these advances have contributed to better understanding of the immunopathogenesis of myositis-associated interstitial lung disease that should ultimately translate into more effective treatment.

Topics: Bronchoalveolar Lavage; Dermatomyositis; Humans; Keratins; Lung; Lung Diseases, Interstitial; Polymyositis; Tomography, X-Ray Computed

It has been suggested that cytokeratin 19 is expressed in regenerated bronchoepithelial cells in patients with pulmonary fibrosis, and serum cytokeratin 19 fragment is elevated in patients with pulmonary fibrosis. We hypothesized that serum antibodies to cytokeratin 19 may be formed in patients with pulmonary fibrosis. To prove the existence of anti-cytokeratin 19 antibodies in patients' sera, human recombinant cytokeratin 19 was stained with patients' sera by a Western immunoblot. Then, we tried to establish an enzyme-linked immunosorbent assay to quantitate anti-cytokeratin 19 antibody in the sera of patients with idiopathic pulmonary fibrosis (IPF) and pulmonary fibrosis associated with collagen vascular disorders (PF-CVD). We demonstrated the anti-cytokeratin 19 antibody in patient' sera by a Western immunoblot. In patients with IPF and PF-CVD, significantly high anti-cytokeratin 19 antibody was demonstrated compared with normal volunteers, patients with chronic bronchitis, and patients with pneumonia. These results suggest that anti-cytokeratin 19 antibody may have played a role in the process of lung injury in pulmonary fibrosis.

Topics: Adult; Aged; Aged, 80 and over; Arthritis, Rheumatoid; Autoantibodies; Blotting, Western; Collagen Diseases; Dermatomyositis; Enzyme-Linked Immunosorbent Assay; Female; Humans; Immunoenzyme Techniques; Keratins; Male; Middle Aged; Pulmonary Fibrosis; Sjogren's Syndrome

Cytokeratin 19 fragment (CK19) levels in serum have been documented as a useful tumor marker for lung cancer. We hypothesize that CK19 may increase in serum in patients with interstitial pneumonia associated with polymyositis/dermatomyositis (PM/DM) and CK19 might be a useful variable to evaluate the activity of lung injury.. 1. We measured CK19 in sera in 15 patients diagnosed with PM/DM; 6 had nonspecific interstitial pneumonia (NIP), 4 had acute interstitial pneumonia (AIP), and 5 had no pulmonary involvement. We also measured CK19 in 10 healthy nonsmokers serving as a control group. 2. We evaluated the correlation between CK19 level and individual clinical course in patients with pulmonary involvement associated with PM/DM.. CK19 levels in sera of patients with NIP associated with PM/DM were significantly higher versus patients with PM/DM without interstitial pneumonia and healthy nonsmokers. CK19 levels in sera in patients with AIP associated with PM/DM were significantly higher compared with the other groups. CK19 values in sera changed according to the progression or improvement of interstitial pneumonia. Immunohistochemical studies using pulmonary tissues obtained at autopsy from patients with AIP associated with PM/DM revealed that the hyaline membrane was mostly stained by anti-human cytokeratin 19 monoclonal antibody as well as the strong positivity of proliferating type II pneumocytes.. These results suggest that the measurement of CK19 was a useful variable to evaluate the activity of lung injury in interstitial pneumonia associated with PM/DM.

Topics: Adult; Aged; Dermatomyositis; Female; Humans; Immunohistochemistry; Keratins; Lung Diseases, Interstitial; Male; Middle Aged; Peptide Fragments

The role of transforming growth factor beta 2 (TGF-beta 2) in the pathogenesis of systemic sclerosis (SSc) was investigated by in situ hybridization of skin biopsies from six patients with SSc. Two patients with acute systemic lupus erythematosis (SLE), one with acute dermatomyositis (DM), and three healthy individuals were used as controls. TGF-beta 2 mRNA was found to be co-localized with pro alpha 1(I) collagen expression around dermal blood vessels in all patients with the inflammatory stage of SSc, whereas there was no expression of either gene in the dermis of patients in the fibrotic stage, the SLE patients or the normal controls. These findings provide evidence that TGF-beta 2 released by inflammatory cells around blood vessels may play a role in mediating the collagen gene disregulation in fibrosis.

Topics: Blood Vessels; Blotting, Northern; Dermatomyositis; Gene Expression; Humans; Keratins; Lupus Erythematosus, Systemic; Nucleic Acid Hybridization; Procollagen; RNA Probes; RNA, Messenger; Sclerosis; Skin Physiological Phenomena; Transforming Growth Factors

Skin eruptions with features of subacute cutaneous lupus erythematosus occurred in a patient with dermatomyositis who was taking griseofulvin. The patient had antibodies against SSA/Ro and SSB/La antigens. Skin rashes disappeared after discontinuation of griseofulvin and recurred on challenge with the drug. These findings support previous studies that the photoactive drug may be synergistic with anti-SSA/Ro antibody to produce lesions of subacute cutaneous lupus erythematosus.

Topics: Adult; Biopsy; Dermatomyositis; Drug Eruptions; Female; Griseofulvin; Humans; Keratins; Lupus Erythematosus, Cutaneous; Skin