Evolution of 65 Patients with Undifferentiated Connective Tissue Disease in a Five-Year Follow-up Study

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Evolution of 65 Patients with Undifferentiated Connective Tissue Disease in

a Five-Year Follow-up Study

Category:  31 Miscellaneous rheumatic diseases

Ivan Ferraz-Amaro, Trujillo, Arantzazu Arteaga, Alberto Alvarez,

J Bethencourt, C Quevedo, Sagrario Bustabad, Federico -,

n Gantes, Tomas

Servicio de Reumatologia. Hospital Universitario de Canarias, Santa Cruz de

Tenerife, Spain

Presentation Number: 76

Poster Board Number: 76

Keywords: undifferentiated, connective tissue disease

Objective.- Evaluate the clinical and serological profiles, as well as the

natural history of 65 patients with undifferentiated connective tissue

disease who had been followed for an average time of 5 years.

Methods.- The retrospective analysis was based on sixty-five patients (61

females and 4 males) with undifferentiated connective disease defined as

meeting at least 3 of 11 specific manifestations of connective tissue

disease and presenting at least one non-organ-specific autoantibody, that do

not fit, at the onset of the symptoms, into any well-known diagnosis or

classification criteria. Patients were evaluated for clinical and

immunological manifestations, death rates, disease remissions, major organ

damage and evolution into one definite connective tissue disease.

Results.- Mean age at onset was 37.68 years (range 18-70). Thirty-four

patients (52%) complained at the onset of articular features, 11 (16%) of

mucocutaneos manifestations, 8 (12%) had haematological findings and 4 (6%)

of Raynaud¹s phenomenon. During the follow-up immunological features

included: 100% of patients were positive for ANA (12% La+, 13% Ro+, 00.3%

Sm+ and 0.01% RNP+); hypocomplementemia in 17 (26%) of patients;

anticardiolipins antibodies in 10 (15%) of patients; leucopenia and

thrombopenia was found in 20% of them and hypergammaglobulinemia in 14

(21%). Most frequent clinical features during 5 years of follow-up were:

arthritis and arthralgias (40%), butterfly rash (10%), photosensitivity

(9%), oral aphthae (13%), alopecia (13%) and sicca complex (16%). Four

patients underwent salivary gland biopsy without specific findings, 17 skin

biopsy (lupus band test positive in 6 patients), 15 salivary gland

scintigraphy (positive in 5) and four patients had altered esophageal

manometry. In five patients (7%) a final diagnosis was reached after 5 years

of follow-up, 3 systemic erythematosus lupus and 2 Sjogren¹s syndrome.

Cloroquine or corticoids were used in 13 patients (20%) most of them because

musculoskeletal complaints.

Conclusion.- In our study we have found a low rate of evolution into a

defined autoimmune disease (7%) in patients with undifferentiated connective

tissue disease. Most of the patients presented a favourable course with a

low rate of major organ involvement and a limited use of immunosupresive

therapy (20%). No clinical and laboratory feature can predict, in our study,

the subsequent occurrence of a definite connective tissue disease.