Undifferentiated connective tissue disease natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

The symptoms of undifferentiated connective tissue disease (UCTD) usually are similar to connective tissue diseases but couldn’t complete the criteria for their definitive diagnosis. If left untreated, 24.3-33% of patients with UCTD may progress to develop manifestations of well defined connective tissue disease such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome. Common complications of UCTD include Interstitial lung disease– Non specific interstitial pneumonia, atherosclerosis. Depending on the extent of the UCTD at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. The presence of interstitial lung disease is associated with a particularly good prognosis among patients with UCTD rather than being idiopathic.

Natural History, Complications, and Prognosis

Natural History

The symptoms of undifferentiated connective tissue disease (UCTD) usually are similar to connective tissue diseases but couldn’t complete the criteria for their definitive diagnosis.
If left untreated, 24.3-33% of patients with UCTD may progress to develop manifestations of well defined connective tissue disease such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome.^[1]^[2]

Complications

Common complications of UCTD include:^[3]
- Interstitial lung disease–
- Non specific interstitial pneumonia
- Atherosclerosis^[4]

Prognosis

Depending on the extent of the UCTD at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.
The presence of interstitial lung disease is associated with a particularly good prognosis among patients with UCTD rather than being idiopathic.^[3]

References

↑ Danieli MG, Fraticelli P, Salvi A, Gabrielli A, Danieli G (1998). “Undifferentiated connective tissue disease: natural history and evolution into definite CTD assessed in 84 patients initially diagnosed as early UCTD”. Clin. Rheumatol. 17 (3): 195–201. PMID 9694051.
↑ Cavazzana I, Franceschini F, Belfiore N, Quinzanini M, Caporali R, Calzavara-Pinton P, Bettoni L, Brucato A, Cattaneo R, Montecucco C (2001). “Undifferentiated connective tissue disease with antibodies to Ro/SSa: clinical features and follow-up of 148 patients”. Clin. Exp. Rheumatol. 19 (4): 403–9. PMID 11491495.
↑ ^3.0 ^3.1 Kinder BW, Shariat C, Collard HR, Koth LL, Wolters PJ, Golden JA, Panos RJ, King TE (April 2010). “Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function”. Lung. 188 (2): 143–9. doi:10.1007/s00408-009-9226-7. PMC 2837880. PMID 20069430.
↑ Laczik, R.; Soltesz, P.; Szodoray, P.; Szekanecz, Z.; Kerekes, G.; Paragh, G.; Rajnavolgyi, E.; Abel, G.; Szegedi, G.; Bodolay, E. (2014). “Impaired endothelial function in patients with undifferentiated connective tissue disease: a follow-up study”. Rheumatology. 53 (11): 2035–2043. doi:10.1093/rheumatology/keu236. ISSN 1462-0324.

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[pmid9694051-1] Danieli MG, Fraticelli P, Salvi A, Gabrielli A, Danieli G (1998). “Undifferentiated connective tissue disease: natural history and evolution into definite CTD assessed in 84 patients initially diagnosed as early UCTD”. Clin. Rheumatol. 17 (3): 195–201. PMID 9694051.

[pmid11491495-2] Cavazzana I, Franceschini F, Belfiore N, Quinzanini M, Caporali R, Calzavara-Pinton P, Bettoni L, Brucato A, Cattaneo R, Montecucco C (2001). “Undifferentiated connective tissue disease with antibodies to Ro/SSa: clinical features and follow-up of 148 patients”. Clin. Exp. Rheumatol. 19 (4): 403–9. PMID 11491495.

[pmid20069430-3] 3.0 ^3.1 Kinder BW, Shariat C, Collard HR, Koth LL, Wolters PJ, Golden JA, Panos RJ, King TE (April 2010). “Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function”. Lung. 188 (2): 143–9. doi:10.1007/s00408-009-9226-7. PMC 2837880. PMID 20069430.

[LaczikSoltesz2014-4] Laczik, R.; Soltesz, P.; Szodoray, P.; Szekanecz, Z.; Kerekes, G.; Paragh, G.; Rajnavolgyi, E.; Abel, G.; Szegedi, G.; Bodolay, E. (2014). “Impaired endothelial function in patients with undifferentiated connective tissue disease: a follow-up study”. Rheumatology. 53 (11): 2035–2043. doi:10.1093/rheumatology/keu236. ISSN 1462-0324.

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