eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease

Undifferentiated Connective-Tissue Disease: Follow-up

Author: Bernard A Hildebrand Jr, MD, Staff Rheumatologist, Department of Rheumatology, San Antonio Military Medical Center, San Antonio, Texas
Coauthor(s): Daniel F Battafarano, DO, FACP, FACR, Clinical Professor of Medicine, University of Texas Health Science Center, San Antonio, Texas; Associate Professor, Department of Medicine, Uniformed Services University of Health Sciences, F Edward Hebert School of Medicine; Chief of Rheumatology Service, San Antonio Military Medical Center, San Antonio, Texas.
Contributor Information and Disclosures

Updated: Jul 24, 2009

Follow-up

Further Inpatient Care

  • Inpatient care in patients with undifferentiated connective-tissue disease (UCTD) may be necessary only for complex/urgent diagnostic evaluations or therapeutic reasons.

Further Outpatient Care

  • Patients with UCTD are typically monitored for progression of specific manifestations, evolution to a defined CTD, and safety and efficacy of treatment. The frequency of outpatient visits necessary depends on the severity of disease. During the first 5 years postdiagnosis, the frequency of follow-up visits may be more frequent given the propensity for UCTD to evolve early in the disease course.

Inpatient & Outpatient Medications

  • Medications are prescribed, adjusted, and monitored as indicated for symptoms and safe medical therapy.

Transfer

  • Transfer of care ultimately depends on any complications of UCTD that might require subspecialty medical or surgical care.

Deterrence/Prevention

  • Deterrence and prevention of UCTD is directly related to the organ or organs involved. However, appropriate preventive medicine screening and immunizations should be considered annually.

Complications

  • The complications of UCTD are related directly to specific organ involvement, susceptibility to infections, drug intolerance/reactions, and drug interactions.

Prognosis

  • The prognosis of UCTD depends primarily on the extent of organ involvement.
  • UCTD may evolve into a defined CTD in 20%-40% of patients with UCTD, while 50%-60% of cases remain undifferentiated.17
  • Approximately 10%-20% of patients with UCTD have symptoms that subside or remit and never evolve into a defined CTD.22 The incidence of evolution to a defined CTD is highest during the first 3-5 years of the disease course,23 and the rate of evolution continues to decrease over time.24
  • Patients who develop a defined CTD late have been noted to have milder disease with a lower incidence of serious adverse events and a better prognosis.15,24
  • Survival rates in UCTD are similar to those associated with RA and SLE.32

Patient Education

  • Patients with UCTD, as well as their immediate family members, should be educated about the prognosis of UCTD and the potential for the disease to evolve into a defined CTD (eg, SLE, SSc, MCTD).
  • Furthermore, education should focus on symptomatic treatment, an appropriate understanding of treatment medications, and the indications for contacting the primary care physician or rheumatologist.

Miscellaneous

Medicolegal Pitfalls

  • Failure to refer a patient with undifferentiated connective-tissue disease (UCTD) to a rheumatologist for diagnosis or treatment if unresponsive to initial therapy
  • Failure to monitor or recognize the complications of UCTD

Special Concerns

Pregnancy

Pregnant patients with newly diagnosed UCTD are at increased risk of impaired intrauterine growth, prematurity, and delivery of infants small for gestational age. The high prevalence of anti-Ro (SSA) antibodies in patients with UCTD suggests an increased potential for neonatal lupus syndrome and congenital cardiac conduction abnormalities.41

 


More on Undifferentiated Connective-Tissue Disease

Overview: Undifferentiated Connective-Tissue Disease
Differential Diagnoses & Workup: Undifferentiated Connective-Tissue Disease
Treatment & Medication: Undifferentiated Connective-Tissue Disease
Follow-up: Undifferentiated Connective-Tissue Disease
References
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References

  1. Arnett FC, Edworthy SM, Bloch DA, McShane DJ, Fries JF, Cooper NS. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum. Mar 1988;31(3):315-24. [Medline].

  2. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. Nov 1982;25(11):1271-7. [Medline].

  3. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. Sep 1997;40(9):1725. [Medline].

  4. American Rheumatism Association. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. May 1980;23(5):581-90. [Medline].

  5. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. Feb 13 1975;292(7):344-7. [Medline].

  6. Alarcón-Segovia D, Cardiel MH. Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients. J Rheumatol. Mar 1989;16(3):328-34. [Medline].

  7. Vitali C, Bombardieri S, Moutsopoulos HM, Balestrieri G, Bencivelli W, Bernstein RM. Preliminary criteria for the classification of Sjögren's syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum. Mar 1993;36(3):340-7. [Medline].

  8. Fox RI, Saito I. Criteria for diagnosis of Sjogren's syndrome. Rheum Dis Clin North Am. May 1994;20(2):391-407. [Medline].

  9. LeRoy EC, Maricq HR, Kahaleh MB. Undifferentiated connective tissue syndromes. Arthritis Rheum. Mar 1980;23(3):341-3. [Medline].

  10. Mosca M, Neri R, Bombardieri S. Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria. Clin Exp Rheumatol. Sep-Oct 1999;17(5):615-20. [Medline].

  11. Danieli MG, Fraticelli P, Franceschini F, et al. Five-year follow-up of 165 Italian patients with undifferentiated connective tissue diseases. Clin Exp Rheumatol. Sep-Oct 1999;17(5):585-91. [Medline].

  12. Greer JM, Panush RS. Incomplete lupus erythematosus. Arch Intern Med. Nov 1989;149(11):2473-6. [Medline].

  13. Ganczarczyk L, Urowitz MB, Gladman DD. "Latent lupus". J Rheumatol. Apr 1989;16(4):475-8. [Medline].

  14. Swaak AJ, van de Brink H, Smeenk RJ, Manger K, Kalden JR, Tosi S. Incomplete lupus erythematosus: results of a multicentre study under the supervision of the EULAR Standing Committee on International Clinical Studies Including Therapeutic Trials (ESCISIT). Rheumatology (Oxford). Jan 2001;40(1):89-94. [Medline].

  15. Bodolay E, Csiki Z, Szekanecz Z, et al. Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD). Clin Exp Rheumatol. May-Jun 2003;21(3):313-20. [Medline].

  16. Cavazzana I, Franceschini F, Belfiore N, et al. Undifferentiated connective tissue disease with antibodies to Ro/SSa: clinical features and follow-up of 148 patients. Clin Exp Rheumatol. Jul-Aug 2001;19(4):403-9. [Medline].

  17. Mosca M, Tani C, Bombardieri S. A case of undifferentiated connective tissue disease: is it a distinct clinical entity?. Nat Clin Pract Rheumatol. Jun 2008;4(6):328-32. [Medline].

  18. Doria A, Mosca M, Gambari PF, Bombardieri S. Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both?. J Rheumatol. Feb 2005;32(2):213-5. [Medline].

  19. Bizzaro N, Tozzoli R, Shoenfeld Y. Are we at a stage to predict autoimmune rheumatic diseases?. Arthritis Rheum. Jun 2007;56(6):1736-44. [Medline].

  20. Horvath L, Czirjak L, Fekete B, et al. Levels of antibodies against C1q and 60 kDa family of heat shock proteins in the sera of patients with various autoimmune diseases. Immunol Lett. Jan 1 2001;75(2):103-9. [Medline].

  21. Spain TA, Sun R, Gradzka M, et al. The transcriptional activator Sp1, a novel autoantigen. Arthritis Rheum. Jun 1997;40(6):1085-95. [Medline].

  22. Szodoray P, Nakken B, Barath S, Gaal J, Aleksza M, Zeher M. Progressive divergent shifts in natural and induced T-regulatory cells signify the transition from undifferentiated to definitive connective tissue disease. Int Immunol. Aug 2008;20(8):971-9. [Medline].

  23. Mosca M, Tani C, Bombardieri S. Defining undifferentiated connective tissue diseases: a challenge for rheumatologists. Lupus. 2008;17(4):278-80. [Medline].

  24. Mosca M, Neri R, Bencivelli W, Tavoni A, Bombardieri S. Undifferentiated connective tissue disease: analysis of 83 patients with a minimum followup of 5 years. J Rheumatol. Nov 2002;29(11):2345-9. [Medline].

  25. Gupta D, Roppelt H, Bowers B, Kunz D, Natarajan M, Gruber B. Successful remission of thrombotic thrombocytopenic purpura with rituximab in a patient with undifferentiated connective tissue disorder. J Clin Rheumatol. Apr 2008;14(2):94-6. [Medline].

  26. Martorell EA, Hong C, Rust DW, Salomon RN, Krishnamani R, Patel AR. A 32-year-old woman with arthralgias and severe hypotension. Arthritis Rheum. Nov 15 2008;59(11):1670-5. [Medline].

  27. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease?. Am J Respir Crit Care Med. Oct 1 2007;176(7):691-7. [Medline].

  28. Deng XL, Liu XY. Comparative Study of 181 Cases of Diffuse Connective Tissue Disease Complicated by Cardiovascular Disease. International Journal of Rheumatic Diseases. Nov 2008;11 (Suppl. 1):A462.

  29. Oh CW, Lee SH, Heo EP. A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease. Am J Dermatopathol. Oct 2003;25(5):423-7. [Medline].

  30. Oishi Y, Arai M, Kiraku J, Doi H, Uchiyama T, Hasegawa A. Unclassified connective tissue disease presenting as cardiac tamponade: a case report. Jpn Circ J. Aug 2000;64(8):619-22. [Medline].

  31. Zhang Y, Zhang FK, Wu XN, Wang TL, Jia JD, Wang BE. Undifferentiated connective tissue diseases-related hepatic injury. World J Gastroenterol. May 7 2008;14(17):2780-2. [Medline].

  32. Williams HJ, Alarcon GS, Joks R, et al. Early undifferentiated connective tissue disease (CTD). VI. An inception cohort after 10 years: disease remissions and changes in diagnoses in well established and undifferentiated CTD. J Rheumatol. Apr 1999;26(4):816-25. [Medline].

  33. Alarcón GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE. Early undifferentiated connective tissue disease. I. Early clinical manifestation in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease. J Rheumatol. Sep 1991;18(9):1332-9. [Medline].

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

  35. Calvo-Alen J, Alarcon GS, Burgard SL, et al. Systemic lupus erythematosus: predictors of its occurrence among a cohort of patients with early undifferentiated connective tissue disease: multivariate analyses and identification of risk factors. J Rheumatol. Mar 1996;23(3):469-75. [Medline].

  36. Heinlen LD, McClain MT, Merrill J, Akbarali YW, Edgerton CC, Harley JB. Clinical criteria for systemic lupus erythematosus precede diagnosis, and associated autoantibodies are present before clinical symptoms. Arthritis Rheum. Jul 2007;56(7):2344-51. [Medline].

  37. Mosca M, Baldini C, Bombardieri S. Undifferentiated connective tissue diseases in 2004. Clin Exp Rheumatol. Jan-Feb 2004;17(5):22(3 Suppl 33). [Medline].

  38. Ceribelli A, Cavazzana I, Franceschini F, Quinzanini M, Rizzini FL, Cattaneo R. Isotype switching and titer variation of anti-Ro/SSA antibodies over time in 100 patients with undifferentiated connective tissue disease (UCTD). Clin Exp Rheumatol. Jan-Feb 2008;26(1):117-20. [Medline].

  39. Zold E, Szodoray P, Gaal J, Kappelmayer J, Csathy L, Gyimesi E. Vitamin D deficiency in undifferentiated connective tissue disease. Arthritis Res Ther. 2008;10(5):R123. [Medline].

  40. Cortes S, Clemente-Coelho P. [Nailfold capillaroscopy in the evaluation of Raynaud's phenomenon and undifferentiated connective tissue disease]. Acta Reumatol Port. Apr-Jun 2008;33(2):203-9. [Medline].

  41. [Best Evidence] Spinillo A, Beneventi F, Epis OM, Montanari L, Mammoliti D, Ramoni V. The effect of newly diagnosed undifferentiated connective tissue disease on pregnancy outcome. Am J Obstet Gynecol. Dec 2008;199(6):632.e1-6. [Medline].

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Further Reading

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Keywords

undifferentiated connective-tissue disease, UCTD, undifferentiated connective-tissue syndromes, UCTS, early connective-tissue disease, rheumatoid arthritis, RA, systemic lupus erythematosus, SLE, systemic sclerosis, SSc, polymyositis, PM, dermatomyositis, DM, mixed connective-tissue disease, MCTD, Sjögren's syndrome, Sjögren syndrome, SS

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Contributor Information and Disclosures

Author

Bernard A Hildebrand Jr, MD, Staff Rheumatologist, Department of Rheumatology, San Antonio Military Medical Center, San Antonio, Texas
Bernard A Hildebrand Jr, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Daniel F Battafarano, DO, FACP, FACR, Clinical Professor of Medicine, University of Texas Health Science Center, San Antonio, Texas; Associate Professor, Department of Medicine, Uniformed Services University of Health Sciences, F Edward Hebert School of Medicine; Chief of Rheumatology Service, San Antonio Military Medical Center, San Antonio, Texas.
Daniel F Battafarano, DO, FACP, FACR is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and Association of Military Surgeons of the US
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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