Undifferentiated Connective-Tissue Disease Treatment & Management

Updated: Jun 15, 2021
  • Author: Bernard Hildebrand, MD, MA; Chief Editor: Herbert S Diamond, MD  more...
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Approach Considerations

A patient with undifferentiated connective-tissue disease (UCTD) can be evaluated and treated primarily as an outpatient. Nonsteroidal anti-inflammatory drugs (NSAIDs), antimalarials (eg, hydroxychloroquine), and corticosteroids are the mainstay of therapy. Immunosuppressive drugs are generally reserved for treating specific clinical manifestations and when there is major organ involvement. In the INBUILD trial of patients with progressive fibrosing interstitial lung diseases, including cases that occurred in the context of UCTD, treatment with the antifibrotic agent nintedanib significantly slowed the rate of decline in forced vital capacity. [57]

Surgery for patients with UCTD is not routinely necessary and should be initiated only when indicated for diagnosis or treatment.




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  • A rheumatologist should be consulted.

  • Consultations with other specialists may be clinically indicated, including with a dermatologist, ophthalmologist, pulmonologist, cardiologist, neurologist, physical medicine specialist, physical therapist, and/or occupational therapist.



See the list below:

  • In general, activities are not restricted in the absence of specific functional limitations associated with UCTD.

  • Patients with photosensitivity should minimize prolonged exposure to sunlight and should use protective clothing and sunblock lotions/creams to protect against ultraviolet light.

  • Patients with severe Raynaud phenomenon should avoid prolonged exposure to severe cold temperatures (< 40° F) to avoid digital vasospasm. Layered clothing, hats, and gloves help to maintain a warm core body temperature and decreased vasospastic symptoms. Tobacco use should be avoided.