Undifferentiated connective tissue disease (UCTD), undifferentiated connective tissue disease (UCTD)
Severe pulmonary manifestation (frostbite changes in the lower part of both lungs) (B) and enlarged lymph nodes (a). Lunardi F, Diagn Patol 2011. CC BY 2.0
Contents
Definition
Many with obvious autoimmune" Inflammatory rheumatic disease cannot be defined (classified) in a particular diagnosis. This is because the disease has symptoms and findings from two or more conditions without a single diagnosis being adequate. Early in the course of the disease, yet unclear symptoms may contribute to more Systemic connective tissue diseases: are difficult to classify. However, undifferentiated systemic connective tissue disease will often remain unclassified (in about 80%) even after the entire course of the disease.
Undifferentiated systemic connective tissue disease is thus distinguished from the following:
- Systemic lupus erythematosus (SLE)
- MCTD (mixed connective tissue disease)
- Sjögren's syndrome
- Systemic Sclerosis
- Myositis (Polymyositis, Dermatomyositis, Inclusion Legemyositis)
- Antisynthetase syndrome
- Scleromyositis (overlap systemic sclerosis and myositis with "own" typical antibodies)
- Rheumatoid arthritis
- Spondyloarthritis
Occurrence
About 25% of systemic connective tissue disease remains unclassified.
Symptoms (a selection) of undifferentiated systemic connective tissue disease
- Raynaud's phenomenon
- Arthritis
- Swollen ("puffy") fingers
- Eczema (dermatitis)
- Hair loss (alopecia)
- Dry eyes and mouth
- Manifestation in various internal organs
- Lungs
- Kidneys
- Heart
- Antibodies of different types at the same time that can accommodate multiple diseases (controlled over time)
- ANA (antinuclear antibody) with different combinations of subgroups, often including SSA / Ro52)
- Anti-CCP without classic rheumatoid arthritis
- In blood samples, the number of blood cells may be low (leukopenia, thrombocytopenia, anemia). However, it is unusual with the low number of all types at the same time (investigate bone marrow for other illness)
- Capillaroscopy can show signs of disease in small blood vessels
- Effect of immunosuppressive drugs
Diagnosis
One wants to describe the symptoms, investigation findings and the course of the disease so that one can map these a clear, unambiguous diagnosis. It is then easier to inform about the condition and follow-up can follow diagnosis-specific recommendations. In undifferentiated systemic connective tissue disease, the individual variation is large within the same condition. One is based on the individual's symptoms and examination findings and describes the individual case.
Incorrect diagnosis (similar conditions, differential diagnoses)
- Other Autoimmune diseases
- Cryoglobulinemia
- Auto-inflammatory disease / Periodic fever syndrome
- Inflammatory bowel disease (Ulcerative colitis, Crohn's disease)
- Painful conditions like Fibromyalgia with at the same time elevated ANA without any other signs Autoimmune disease
The treatment
An individually adapted treatment is at least as relevant as with other systemic connective tissue diseases. Some do not need medication, while in others fixed use of potent drugs can prevent serious organ damage.
Prognosis
In general, undifferentiated systemic connective tissue disease has a good prognosis because injuries to internal organs rarely occur. However, the course is characterized by the individual symptoms.
