Undifferentiated connective tissue diseases: the clinical and serological profiles of 91 patients followed for at least 1 year

Mosca, Marta; Tavoni, Antonio; Neri, R; Bencivelli, W.; Bombardieri, Stefano

doi:10.1191/096120398678919787

Cited by 98 publications

(68 citation statements)

References 18 publications

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“…The published prospective studies [12][13][14][15][16][17][18] found an evolving frequency that varies from 5% to 68% of patients. According to this data, it is estimated that 20-40% of patients with early UCTD will develop a definite CTD [11].…”

Section: Discussionmentioning

confidence: 99%

Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients

Vaz

Couto

Medeiros³

et al. 2009

Clin Rheumatol

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The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). Consecutive patients with UCTD, followed up at the Rheumatology Clinic of the participating centers, were included. Data from these patients were obtained by clinical evaluation and chart review. All patients were diagnosed as having UCTD on basis of the following criteria: positive ANA plus at least one clinical feature of connective tissue disease, but not fulfilling classification criteria for any differentiated connective tissue disease. One hundred eighty-four patients were studied (female patients-94.5%; mean age at time of evaluation-47 years). The most prevalent manifestations were arthralgia (66%), arthritis (32%), Raynaud's phenomenon (30%), sicca symptoms (30%), and leukopenia (19%). The prevalence of ANA was 100%, anti-SSA 20%, antidsDNA 14%, and anti-SSB 7%. Patients with anti-dsDNA/ anti-Sm, anticentromere/anti-Scl70, or anti-SSA/anti-SSB antibodies more frequently presented a set of manifestations close to systemic lupus erythematosus (SLE), systemic sclerosis, or Sjögren syndrome, respectively. We analyze a large cohort of UCTD. Seventy-two percent of these UCTD patients present lupus-, scleroderma-, or Sjögren-like features but do not fulfill classification criteria and mostly present a mild disease.

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Section: Discussionmentioning

confidence: 99%

Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients

Vaz

Couto

Medeiros³

et al. 2009

Clin Rheumatol

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“…The study population consisted of 57 Caucasian patients with the following connective tissue diseases: Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), diagnosed according to the criteria of the American College of Rheumatology (formerly, the American Rheumatism Association) and the European Community Study Group (11)(12)(13), undifferentiated connective tissue disease (UCTD) (14), and mixed connective tissue disease (MCTD) (15). Patients were grouped according to positivity (n ϭ 31) and negativity (n ϭ 26) for anti-Ro/SSA antibodies.…”

Section: Methodsmentioning

confidence: 99%

Prolongation of the corrected QT interval in adult patients with anti‐Ro/SSA–positive connective tissue diseases

Lazzerini¹,

Acampa²,

Guideri³

et al. 2004

Arthritis & Rheumatism

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Objective. Newborns of mothers positive for antiRo/SSA autoantibodies may develop a series of electrocardiographic (EKG) disturbances. Prolongation of the corrected QT (QTc) interval was recently reported in a significant proportion of children with maternally acquired anti-Ro/SSA antibodies, with a concomitant disappearance of EKG abnormalities and acquired maternal autoantibodies during the first year, suggesting a direct, reversible electrophysiologic effect of anti-Ro/ SSA antibodies on the ventricular repolarization. On this basis, we investigated whether these antibodies may also affect cardiac repolarization in anti-Ro/SSApositive adult patients with connective tissue diseases.Methods. Fifty-seven patients with connective tissue diseases were selected: 31 had anti-Ro/SSA antibodies and 26 did not (controls). In all subjects, we analyzed the QTc interval, heart rate variability, and signalaveraged high-resolution EKG recording.Results. Anti-Ro/SSA-positive patients showed a significant prolongation of the mean QTc interval compared with the controls (mean ؎ SD 445 ؎ 21 versus 419 ؎ 17 msec; P ‫؍‬ 0.000005). Eighteen of the 31 anti-Ro/SSA-positive patients (58%) and none of the 26 anti-Ro/SSA-negative patients had QTc values above the upper limit of normal (440 msec). Both groups had a reduction in heart rate variability, with a prevalence for the sympathetic nervous system and a high incidence of ventricular late potentials; these values were not significantly different between the 2 groups.Conclusion. Adult patients with anti-Ro/SSApositive connective tissue diseases showed a high prevalence of QTc interval prolongation. This feature, with the concomitant abnormalities in the autonomic tone and ventricular late excitability observed in all patients studied, suggests that anti-Ro/SSA-positive patients may have a particularly high risk of developing lifethreatening arrhythmias.Neonatal lupus is a rare syndrome related to the transplacental passage of autoantibodies from mothers positive for anti-Ro/SSA (and anti-La/SSB) to their newborns. These mothers frequently have autoimmune disorders, especially connective tissue diseases; however, they are sometimes entirely asymptomatic. The main feature of neonatal lupus is congenital heart block (CHB), an irreversible disturbance in cardiac conduction. Many recent studies have investigated the possible electrophysiologic and molecular mechanisms of CHB, and the findings suggest a direct arrhythmogenic activity of anti-Ro/SSA antibodies. In particular, it has been demonstrated that affinity-purified anti-Ro/SSA antibodies from mothers of children with CHB are able to induce a complete atrioventricular block in the human fetal heart as well as in a rat model of the heart, which inhibits L-type calcium currents (1).CHB is the main, but not the only, electrocardiographic abnormality associated with the transplacental passage of anti-Ro/SSA antibodies. Mazel and colleagues (2) reported sinus bradycardia in a murine model injected with IgG obtained from mothers of childr...

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“…La popolazione di pazienti in esame è già stata precedentemente descritta (7,14). Si tratta di una coorte di 91 pazienti con diagnosi di UCTD della durata di almeno un anno seguiti presso la nostra Unità Operativa.…”

Section: Materiali E Metodiunclassified

“…Le manifestazioni cliniche e sierologiche presentate dai pazienti sono state raccolte in apposite cartelle cliniche. I dati relativi alle manifestazioni cliniche e sierologiche presenti alla prima osservazione erano già disponibili dalla analisi effettuata in occasione dei precedenti studi (7,14). La ricerca degli autoanticorpi è stata effettutata utilizzando tecniche validate (7,14).…”

Section: Materiali E Metodiunclassified

“…All'esordio di malattia possono essere identificate come UCTD condizioni estremamente eterogenee che comprendono malattie transitorie e autolimitantesi, forme incomplete di connettiviti definite, forme iniziali di connettiviti definite, e, infine, condizioni indifferenziate e indefinibili (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). L'identificazione precoce di tali sottogruppi riveste una notevole importanza pratica e di ricerca ed è stata oggetto di numerosi studi.…”

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

Mosca

Tani²,

Neri³

et al. 2011

Reumatismo

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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

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Undifferentiated connective tissue diseases: the clinical and serological profiles of 91 patients followed for at least 1 year

Cited by 98 publications

References 18 publications

Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients

Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients

Prolongation of the corrected QT interval in adult patients with anti‐Ro/SSA–positive connective tissue diseases

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

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