Showing papers on "Undifferentiated connective tissue disease published in 1996"

Quantitative nailfold capillaroscopy findings in a population with connective tissue disease and in normal healthy controls.

Abstract: OBJECTIVE: To describe and quantify the morphological characteristics of nailfold capillaries that distinguish different forms of connective tissue disease from healthy controls. METHODS: A CCD video microscope with fibreoptic illumination and PC based image processing was used to visualise nailfold capillaries and to quantify findings in 23 patients with systemic sclerosis (SSc), 22 patients with systemic lupus erythematosus (SLE), 21 patients with undifferentiated connective tissue disease (UCTD), and 38 healthy controls. RESULTS: Capillary density was reduced in SSc (5.2 (SD 1.3) capillaries/mm) compared with other patient groups and controls. The average number of enlarged capillaries/finger was high in all disease groups (5.5-6.6) compared with controls (2). However, giant capillaries were most frequent in SSc (43%) and were not present in controls. Mild and moderate avascular areas were present in all groups (35%-68%), but severe avascularity was most frequent in SSc (44%) compared with other patients (18%-19%) and controls (0%). The greatest frequency of extensive haemorrhage was in SSc (35%). CONCLUSIONS: There is a range of abnormal capillary findings in patients with connective tissue disease and healthy controls. However, certain abnormalities such as a reduced number of capillaries, severe avascularity, giant capillaries, and haemorrhage are most commonly associated with SSc. Videomicroscopy with image processing offers many technical advantages that can be exploited in further studies of nailfold capillaries.

Systemic lupus erythematosus: predictors of its occurrence among a cohort of patients with early undifferentiated connective tissue disease: multivariate analyses and identification of risk factors.

Abstract: OBJECTIVE To determine predictors of the occurrence of systemic lupus erythematosus (SLE) in patients with early (< or = 1 yr) undifferentiated connective tissue disease (CTD). METHODS Analysis of a cohort of 213 patients with early undifferentiated CTD at entry, followed for 5 yrs at 11 tertiary centers. Baseline demographic, clinical, and laboratory data were compared using univariate and Cox multivariate regression analyses to identify possible predictive features for the subsequent occurrence of SLE. RESULTS 143 of 213 patients had ascertainable clinical status at 5 yrs. By univariate analyses those who evolved to SLE (13%) were more likely to be younger, African-American, and to have alopecia, serositis, discoid lupus, positive Coombs' test, positive anti-dsDNA and anti-Sm antibodies, positive ANA (homogeneous pattern), and/or a false positive test for syphilis. Discoid lupus (relative risk = 15.8), serositis (4.1), ANA-homogeneous (4.8), and anti-Sm positivity (28.2) were retained as predictors of the occurrence of SLE in the Cox regression model. CONCLUSION Some clinical and laboratory features in patients with early undifferentiated CTD can predict the subsequent occurrence of SLE.

Early undifferentiated connective tissue disease. IV. Musculoskeletal manifestations in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of patients with well‐established connective tissue diseases: Followup analyses in patients with unexplained polyarthritis and patients with rheumatoid arthritis at baseline

Abstract: Objective. To examine the musculoskeletal manifestations in a large cohort of patients (n = 410) diagnosed with either a well-established connective tissue disease (CTD) (n = 197) or an early undifferentiated CTD (n = 213) with a symptom duration of < 1 year. This study was aimed at determining the predictive value of demographic, clinical, and laboratory features on outcome in patients with unexplained polyarthritis (UPA) (from the early undifferentiated CTD cohort; n = 67) or rheumatoid arthritis (RA) (from the well-established CTD cohort; n = 57), over a 5-year followup period. Methods. Patients from both cohorts were assessed at years 1, 3, and 5. At the study visits, clinical data were collected in a standardized manner, and sera were obtained and stored. A priori criteria were established for patient ascertainment and diagnosis over the duration of the study. Standard statistics were used for comparisons of baseline characteristics in patients diagnosed as having systemic lupus erythematosus, RA, undifferentiated CTD, and UPA at entry into the cohorts. Baseline features in patients with UPA were examined according to the different subsequent outcomes (RA, CTD, or undifferentiated CTD, remission [nonpersistent], or persistent or active UPA). Baseline features in patients with RA whose disease remained active versus those in whom remission was attained were also examined. Two multivariable analyses, classification trees and polychotomous logistic regression, were performed to predict disease outcomes over time. Results. The overall rate of ascertainment for the 410 patients ranged from 90% at year 1 to 71% at year 5. Patients with established CTDs showed a tendency for more stable diagnoses than those with early undifferentiated CTDs (90–100% versus 45–70%). Consistent baseline predictors of persistent active disease among patients with RA, in both univariate and multivariable analyses, were higher joint counts for pain and tenderness and higher erythrocyte sedimentation rate (ESR). In ∼20% of patients who were classified as having RA when they originally entered the cohort, the disease was in remission at 5 years. Twenty percent of the patients originally classified as having UPA developed RA over the duration of the study. These patients tended to be older and to have swelling of small joints at baseline. However, a consistent pattern of predictive variables could not be identified in the multivariable analyses, other than at year 1 (higher small joint counts for swelling and higher ESR). Conclusion. Baseline features (joint counts and ESR) among RA patients were variously predictive of persistently active disease at years 1–5. Consistent baseline predictors of outcome among the patients with UPA only emerged at year 1. Remission occurred in ∼20% of RA patients, whereas a similar percentage of patients with UPA developed RA. These findings have implications with regard to treatment decisions in patients with early RA and/or UPA.

Methotrexate in nonrenal lupus and undifferentiated connective tissue disease--a review of 36 patients.

Abstract: Objective To determine the efficacy, tolerability, and steroid sparing effect of methotrexate (MTX) in patients with systemic lupus erythematosus (SLE) in clinical practice. Methods From a database of 467 patients, we identified all patients with SLE and undifferentiated connective tissue disease (UCTD, 2-3 criteria for SLE) who had received MTX. Details of previous therapy, indications for MTX, efficacy, toxicity, and steroid reduction during MTX therapy were recorded. Results 21 patients with SLE who had been treated with MTX were identified. The mean weekly MTX dose rose from 7.5 mg at initiation to 13.6 mg at 6 mo and 17.1 mg at 12 mo. A response was seen in 12 of 21 patients, and 7 patients had a sustained 50% reduction in disease activity at the final evaluation. Response was best in patients with dermatitis (5/6), arthritis (6/13), and myositis (1/1), but minimal in patients with central nervous system dysfunction (1/4), serositis (1/3), and isolated fatigue (0/1). Toxicity was noted in 62% of patients, but only 33% discontinued due to toxicity. MTX was continued in 74% of patients at 6 mo and 40% at 12 mo. Steroid dosage was reduced to half the original dose in 9 of 16 patients. A similar pattern of MTX efficacy and toxicity was observed in 15 patients with UCTD. Conclusion MTX is useful in the treatment of some patients with mild manifestations of SLE, with an acceptable toxicity profile, but only modest steroid sparing potential. Patients with dermatitis and arthritis appear to have the best chance of responding to MTX therapy.

Long term follow-up of children with mixed connective tissue disease.

Abstract: Mixed connective tissue disease (MCTD) is characterized by features of more than one of the rheumatic disorders with antinuclear antibodies in a speckled pattern and with antibodies to nuclear ribonucleoprotein (nRNP). MCTD is uncommon in children and long-term follow-up studies in children are infrequently reported. A retrospective review of clinical experience at five pediatric rheumatology centers provided 11 patients who met the following inclusion criteria: (1) Kasukawa's criteria for MCTD1; (2) presentation younger than 18th birthday; (3) greater than five years of follow-up; (4) completion of data collection form. The widely varying outcomes of these 11 children with MCTD on long-term follow-up may lend doubt that this is a unique and distinctive rheumatologic disorder.

A novel anti-microfilament antibody, anti-135 kD, is associated with Raynaud's disease, undifferentiated connective tissue disease and systemic autoimmune diseases.

Abstract: We report herein the characterization of a human IgG antibody reactive with a nonmuscle 135 kD microfilament-associated protein, anti-135 kD. Using nonmuscle epithelial PtK2 cells as substrate in indirect immunofluorescence, we identified a distinctive pattern of reactivity with microfilaments in sera from 12 of 165 (7.3%) patients investigated for systemic autoimmune diseases and in only 2 of 171 (1.2%) normal and rheumatic disease controls (P < 0.006, 95% CI 1.46 to 30.1). An association between anti-135 kD and Raynaud's phenomenon (n= 12/14, 85.7%) with or without an associated systemic autoimmune disease was noted. The anti-135 kD specificity was established by several criteria. (1) The fluorescence was periodically distributed along microfilaments and concentrated at focal adhesions for all sera (n = 14). (2) On immunoblots, the 14 sera reacted with a PtK2 polypeptide of 135 kD. (3) IgC purified by blot-affinity from the 135 kD band (a-135) reproduced the fluorescent pattern of the original sera whil...