Objective. To examine interrelationships among myositis subsets, autoantibodies, and major histocompatibility complex (MHC) class 1 1 alleles across ethnic lines, and to localize genetic susceptibility (presence of HLA-DR versus DQ) to myositis within the MHC class 1 1 region.Methods. MHC class I1 alleles (HLA-DRBZ, DQM, and DQBI, detected by DNA oligotyping) and myositis-specific autoantibodies (MSA) were determined in 224 patients with various myositis syndromes, including 89 whites, 89 African-Americans, 25 MexicanAmericans, and 21 Japanese.Results. Anti-Jo-1 (histidyl-transfer RNA [tRNA] synthetase) and other MSAs (anti-PL-12, anti-PL-7, anti-OJ, anti-EJ, anti-KJ, anti-tRNA, and antisignal recognition particle) were equally distributed among the races, but occurred more often in patients with polymyositis (PM) than in those with dermatomyositis (DM) or other myositis syndromes. MSA frequencies were significantly positively associated with anti-Ro (SS-A) (P = 0.002), and significantly negatively associated with anti-U1 RNP (P = 0.003). Frequencies of the HLA-DRBI *0301 (DR3), DQAZ*0501, and DQBl*O2OISupported by grants from the Muscular Dystrophy Association, the RGK Foundation, NIH grants R-29AR-39325, AR-32214, and AI-27181, and medical research funds from (DQ2) alleles (and haplotype) were each increased in white patients with myositis, especially those with PM, but most strikingly in those with MSAs. However, in the other ethnic groups, except the Japanese group, only frequencies of HLA-DQAI *0501 and the structurally similar DQAI *0402 alleles were significantly increased. The presence of HLA-D@iI*0501 or *040I was most significantly associated with anti-Jo-1, anti-PLU, and other MSAs, compared with myositis patients without MSAs (P = 0.0008,Pc,, = 0.01, odds ratio [OR] = 3.7), and with normal, ethnically matched controls (P = 3 X lo-', P,,, = 1 x OR = 6.5). Among MSApositive patients who were negative for HLADQAl*O501 and *0401, including Japanese patients, the HLA-D&iI *OI02 and *OI03 alleles predominated. In addition, there appeared to be a negative association of the HLA-DR2 alleles (DRBI*I501 and *1503) with PM (P = 0.007, P,,, not significant, OR = 039), but not with DM or myositis overall.Conclusion. By transracial gene mapping, genetic susceptibility to anti-Jo-1 and other MSAs in patients with myositis can be localized within the MHC region to the HLA-DQ4I locus.Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory myopathies that are characterized clinically by proximal muscle weakness, elevations in the level of serum muscle enzymes, especially creatine kinase (CK), and typical abnormal findings on electromyography. Histopathologic analysis has revealed that affected skeletal muscles are infiltrated by inflammatory mononuclear cells, predominantly CDS+ T lymphocytes, and that those muscles show myofibril degeneration, regeneration, and/or necrosis. Similar clinical and histopathologic features of myositis have been observed in association with malignancies, other connective ti...

Objective. To identify components of the patient-doctor relationship associated with trust in physicians. Methods. We assessed 102 patients with systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA) seen at publicly funded hospitals in Houston, Texas. Patients completed a self-response survey examining patient perceptions of the medical encounter and trust in their physicians. Evaluated components of physicians' behaviors included: informativeness, sensitivity to concerns, reassurance and support, patient-centeredness, and participatory decision-making style. Scales were scored 0 to 10, with higher numbers indicating more positive perceptions of communication.Results. Seventy patients had RA and 32 SLE; 25% were white, 43% Latino, 31% African American, and 75% were female. Mean scores for the medical interaction and trust scales ranged from 6.2-7.1, indicating moderate degrees of positive perceptions. All components were highly and positively correlated with each other, and with trust, suggesting that these traits are all elements of a positive style of doctor-patient communication. In multivariate analysis, ethnicity, physicians' informativeness, physicians' sensitivity to concerns, patient-centeredness, disease activity, and patient trust in the US health care system were independent predictors of trust in physicians. A separate model examined the predictors of patient disclosure of information. Patient perceptions of physicians' patient-centeredness and severity of disease activity were independently predictive of patient disclosure of information. Conclusion. In patients with SLE and RA, trust in physicians is significantly associated with patients' ethnicity and their perceptions about specific components of physicians' communication style. Trust in physicians can be improved by using a patient-centered approach, being sensitive to patient concerns, and providing adequate clinical information. Furthermore, patients appear to be more willing to disclose concerns when physicians use a patient-centered communication style.

Relapsing polychondritis is a rare disease most commonly presenting as inflammation of the cartilage of the ears and nose. Auricular chondritis, with red ears resembling infectious cellulitis, is the most common initial finding. Antibodies to type II collagen in cartilage are found, and the earlobes are classically spared. Chronic disease may result in a flabby, droopy ear, cauliflower ear, or saddle nose deformity. Acute involvement of the tracheal cartilage may cause collapse of the airway with obstruction and pulmonary infections. Arthritis may be oligoarticular or polyarticular, most often involving the costochondral junctions. Other manifestations include audiovestibular damage; heart valve disease; and neurologic, ocular, and renal disease. Corticosteroids remain the major treatment. Other therapies include nonsteroidal anti-inflammatory drugs, dapsone, colchicine, azathioprine, methotrexate, cyclophosphamide, hydroxychloroquine, cyclosporine, and infliximab.

Myositis has been associated with HLA-B8 and DR3, especially in white patients with polymyositis and serum anti-Jo-1 antibodies. Twenty-eight patients with myositis and serum translation-related autoantibodies anti-Jo-1, anti-PL-7, anti-PL-12, anti-KJ, and anti-SRP were studied for HLA class I1 specificities by Southern blotting with HLA-DRP, DQP, and D Q a probes. The association of HLA-DR3 (DRwl7) with anti-Jo-1 antibodies in white myositis patients was confirmed (P = 0.003, relative risk 8.9). However, HLA-DRw52 haplotypes, regardless of subtype, were present in all of the white and black patients with serum anti-Jo-1 and other translation-related autoantibodies. Moreover, one anti-Jo-1 positive patient had HLADRw8, an HLA-DRw52 haplotype on which the DRp3 gene has been partially deleted. No HLA-DQ specificity or allele was common to all patients. The HLA-DR3, DRS, DRw6, and DRw8 haplotypes, which bear the HLA-DRw52 specificity, share the most homology in the DRPl first hypervariable region at amino acid positions 9-13. Thus, this DRPl region appears to be the most likely candidate "epitope" for translation-related autoimmune responses in inflammatory myositis.Inflammatory myositis, particularly polymyositis in adults and dermatomyositis in children, has been associated with HLA-B8 and DR3 in white individuals (1,2). In an earlier study using serologic HLA typing (3), the specific subset of myositis patients with serum anti-histidyl-transfer RNA (tRNA) synthetase (antiJo-1) autoantibodies (4) had an even higher frequency of HLA-DR3 than myositis patients overall; all 11 patients with serum anti-Jo-1 antibodies had either HLA-DR3 and/or HLA-DRw6. It was hypothesized that this autoimmune response in myositis may be

Focal sialadenitis is now widely accepted as an objective criterion for the oral component of Sjogren's syndrome (xerostomia). We investigated the association between labial salivary gland histopathologic changes and the clinical and serologic features of 192 patients with suspected connective tissue disorders. A retrospective review of the medical records of all patients was performed, and historical, physical, laboratory, histologic, and roentgenographic data were abstracted. Each patient had undergone labial salivary gland biopsy as part of a rheumatologic evaluation. There were significant associations between positive findings on lip biopsy and the presence of keratoconjunctivitis sicca (P = 0.013), positive antinuclear antibodies (titer 11:80) (P = 2 x lo-'), and positive Ro antibodies (P = 1 x lo-'). However, sicca symptoms and glandular enlargement were not statistically associated with positive findings on lip biopsy. Features predictive of a positive lip biopsy included Ro antibodies (P = 0.914), keratoconjunctivitis sicca (P = 0.700), and positive antinuclear antibodies (P = 0.590).There is significant clinical heterogeneity among patients with sicca symptoms, ranging from From the