Analysis of the evolution to defined connective tissue diseases of patients with “early unidifferentiated connective tissue diseases (UCTD)”

Abstract

The term undifferentiated connective tissue diseases (UCTD) is used to identify systemic autoimmune diseases not fulfilling classificative criteria for defined connective tissue diseases (CTD). Aim of the present study was to evaluate the evolution to defined CTD of an historical cohort of 91 UCTD patients followed at our Unit and to describe clinical and serological characteristics of stable UCTD patients with a disease duration of more than 5 years. Patients, previously described, were selected for having an undifferentiated profile after 1 year of follow up. These patients have been regularly followed at our Unit and their diagnosis has been reassessed annually based on the existing classificative criteria. Seven UCTD patients with a follow up of less than 5 years have been excluded from the study, therefore 84 patients (F: 81, M: 3) have been analysed. During the follow up 28 patients (33%) developed a defined CTD. In particular 22 patients developed systemic lupus erythematosus (SLE), while the remaining 6 patients developed other CTDs (2 primary Sjögren’s syndrome, 2 overlap syndromes, 1 Systemic Sclerosis, 1 rheumatoid arthritis). The evolution to a defined CTD occurred after a mean disease duration of 80.6± 66.8 months (min 14, max 336, median 72); the evolution to SLE occurred after a mean disease duration of 66.8±43.3 months (min 17, max 216, median 57). Anti-cardiolipin antibodies were the only variable correlated with the evolution to SLE (p<0.05). Stable UCTD were characterized by a simplified clinical picture with no major organ involvement and by a simplified autoantibody profile (anti-Ro/SSA antibodies and anti-RNP antibodies were the single antibody specificities observed in 22% and 13% of patients respectively). These results confirm previous data showing that about 30% of UCTD patients will develop a defined CTD, the predictive role of anti-cardiolipin antibodies for the evolution to SLE, and the existence of stable UCTD, distinct clinical entities with a simplified clinico-serological profile. The early identification of stable UCTD is very important both from a clinical and a research point of view. Future research is needed to define a new set of classification criteria

Development of SLE among “potential SLE” patients seen in consultation: long-term follow-up

“…Malar rash and anticardiolipin antibodies were predictors of developing complete SLE in that Swedish cohort . In contrast, in another study of 84 patients with UCTD in which 22 developed SLE after at least five years of follow‐up, only anticardiolipin antibodies were found to predict development of SLE and another six patients developed other forms of connective tissue disease . Other studies have also identified serositis, alopecia and anti‐dsDNA antibodies as predictors of evolution to SLE .…”

“…Previous studies have investigated the evolution of SLE from ‘incomplete lupus’ (fewer than four ACR criteria) or undifferentiated connective tissue disease (UCTD, patients with clinical, laboratory and serologic features characteristic of more than one rheumatic disease) by identifying clinical and serological predictors of the development of SLE . For example, Vilá LM and colleagues followed a group of 87 patients with ‘incomplete lupus’ for a mean of 2.2 years and found that only 9% evolved to SLE.…”

Fibrillary glomerulonephritis with prevalent IgA deposition associated with undifferentiated connective tissue disease: A case report

“…This case report describes a very rare association between UCTD and renal involvement. This event is defined by some authors as virtually absent [ 3 ]. The patient's signs and symptoms were classified as UCTD.…”

“…In this controversy, we introduce a stimulating contribution to this discussion in a case report where FGN was found to be associated with a peculiar deposition of IgAk, without paraproteinaemia but in the interesting context of an undifferentiated connective tissue disease (UCTD). UCTD is described as a systemic autoimmune disease not completely fulfilling the classificative criteria for a defined CTD [ 3 ]. To our knowledge, renal involvement and, in particular, IgA nephropathy (IgAN) have never been described in the course of UCTD, in spite of the well-known IgAN secondary to rheumatologic or gastroenteric autoimmune diseases.…”

Primary dysmenorrhea in adolescent girls as a predictor of the development of undifferentiated connective tissue dysplasia

“…Подобный разброс свидетельствует о том, что НДСТ остается недостаточно изученной проблемой, что обусловлено ее клинической гетерогенностью, отсутствием единой терминологии, общепринятых критериев диагностики и оценки степени тяжести. В последнее десятилетие интерес к данной проблеме чрезвычайно возрос, что во многом обусловлено модифицирующим и, как правило, негативным влиянием данной патологии на течение, прогноз и лечение самых раз-личных соматических заболеваний, увеличением частоты аллергических и аутоиммунных заболеваний [42].…”