Article

Undifferentiated connective tissue disease in a rheumatology center in Cali, Colombia: clinical features of 94 patients followed for a year.

Department of Internal Medicine, Universidad Libre, Cali, Colombia.
Rheumatology International (impact factor: 1.88). 11/2011; DOI:10.1007/s00296-011-2234-y
Source: PubMed

ABSTRACT The aim of the study is to evaluate the clinical and serological features of patients with undifferentiated connective tissue disease (UCTD) of a rheumatology referral center in Cali, Colombia, who were followed for a year. A retrospective analysis of a cohort of patients with an initial diagnosis of UCTD and monitoring for at least 12 months was carried out. A total of 94 patients with UCTD (97.9% women) were evaluated, with an average follow-up of 51 ± 35.7 months. Only 13 patients (13.8%) evolved into a defined connective tissue disease (CTD), of which 8.5% (n:8) developed systemic lupus erythematosus (SLE), 4.2% (n:4) Sjögren syndrome (SS) and 1.1% (n:1) rheumatoid arthritis (RA). A mean period of 35.8 ± 29.2 months between UCTD diagnosis and definite develop of a CTD was found. Arthritis, Raynaud's phenomenon and photosensitivity were statistically significant (<0.001) for development of CTD. After a mean follow-up of 4.25 years, most of the patients with UCTD showed a favorable evolution. Arthritis, Raynaud's phenomenon and the presence of photosensitivity were predictors for the development of CTD. It requires a consensus to establish criteria for the classification of UCTD.

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    Article: Acute fibrinous and organizing pneumonia and undifferentiated connective tissue disease: a case report.
    Valéria Valim, Roberta Hora Rocha, Roberta Barcelos Couto, Thaysa Simões Paixão, Erica Vieira Serrano
    [show abstract] [hide abstract]
    ABSTRACT: Acute fibrinous and organizing pneumonia (AFOP), recently described, is a histologic pattern characterized by the presence of fibrin "balls" within alveolar spaces. The term undifferentiated connective tissue disease (UCTD) is used to identify autoimmune systemic diseases that do not fulfill the criteria to be classified as a definitive connective tissue disease. The AFOP has never been reported in association with UCTD. The present reported case is a 39-year-old Caucasian, female with dry cough and progressive dyspnea. Eight months later, she was diagnosed with "organizing pneumonia" based on clinical history and radiologic images. She manifested Raynaud's Phenomenon, sicca syndrome, boot and gloves neuropathic pain, and previous hypothyroidism. Antinuclear antibody, rheumatoid factor, and specific autoantibodies were negative. Salivary gland biopsy and electroneuromyiography were normal. The capillaroscopy showed a "scleroderma" pattern with capillary deletion and ectasia. She experienced clinical and radiologic worsening. Despite being submitted to cyclophosphamide pulse, she developed hemorrhage and then died. Thoracotomy pulmonary specimen showed histological pattern of AFOP. This paper shows a rare association of AFOP with UCTD.
    Case reports in rheumatology. 01/2012; 2012:549298.
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Keywords

12 months
 
13 patients
 
94 patients
 
average follow-up
 
Colombia
 
defined connective tissue disease
 
favorable evolution
 
initial diagnosis
 
mean follow-up
 
mean period
 
Raynaud's phenomenon
 
retrospective analysis
 
rheumatology referral center
 
serological features
 
SLE
 
SS
 
systemic lupus erythematosus
 
UCTD
 
UCTD diagnosis
 
undifferentiated connective tissue disease
 

Luis F Guerrero