Undifferentiated connective tissue disease

Last revised by Henry Knipe on 4 Jul 2023

Undifferentiated connective tissue disease (UCTD) is a type of connective tissue disease. UCTD should not be confused with mixed connective tissue disease (MCTD) as these two entities describe patient populations with different characteristics.

UCTD comprises patients who have clinical characteristics and serological markers that are compatible with a systemic connective tissue disease. However, these characteristics are not specific enough for a classification to a more defined disease (e.g. systemic lupus erythematosus, scleroderma, myositis, or rheumatoid arthritis) 1.

In the case of "overlap syndromes", patients show clinical characteristics and serological markers that fit several of the defined diseases. Mixed connective tissue disease (MCTD) is one of these overlap syndromes.

Still, if patients with UCTD are followed over a few years, a part of the them may develop a defined disease, such as rheumatoid arthritis or Sjogren's syndrome 2-4. The remaining patients will remain unclassifiable and organ involvement is less common in this group 1.

If present, imaging findings may hint at one of the underlying more specific diseases that have not yet developed in full:

The first mention of the concept of UCTD was in 1980 by E. Carwile LeRoy and colleagues 5.

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