Undifferentiated connective tissue disease (UCTD) is a disease in which the body mistakenly attacks its own tissues. It is diagnosed when there is evidence of an existing autoimmune condition which does not meet the criteria for any specific autoimmune disease, such as

systemic lupus erythematosus Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Comm ...

or scleroderma. Latent lupus and incomplete lupus are alternative terms that have been used to describe this condition. The term is sometimes used interchangeably with

mixed connective tissue disease Mixed connective tissue disease, commonly abbreviated as MCTD, is an autoimmune disease characterized by the presence of elevated blood levels of a specific autoantibody, now called anti-U1 ribonucleoprotein (RNP) together with a mix of symptoms of ...

, an overlap syndrome. However, MCTD is thought by some researchers to be a clinically distinct entity and is strongly associated with the presence of high titers of ribonucleoprotein (RNP) antibodies. It is estimated that up to 25 percent of people with systemic autoimmune disease could be considered to have UCTD.

Signs and symptoms

Disease presentation varies widely from patient to patient, as UCTD is by definition nonspecific. Symptoms typically include constitutional complaints that are common to connective tissue diseases such as

fatigue Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...

, a general sense of feeling unwell, and

fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...

. Other symptoms associated with UCTD include: * dry eyes * dry mouth * hair loss * joint inflammation * joint pain * oral ulcers * positive ANA test * raynaud's phenomenon * sun sensitive rash Clinical presentation in some people diagnosed with UCTD may show: * a decrease in white blood cell count in the blood *

anemia Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, th ...

* abnormal nerve sensations in the extremities * inflammation of the lining of the heart and/or lungs * a decrease in platelet count Lung involvement, such as

nonspecific interstitial pneumonia Non-specific interstitial pneumonia (NSIP) is a form of idiopathic interstitial pneumonia. Symptoms Symptoms include cough, difficulty breathing, and fatigue. Causes It has been suggested that idiopathic nonspecific interstitial pneumonia ha ...

, is a possible disease complication.

Diagnosis

There is no official diagnostic criteria for UCTD. Diagnostic testing generally aims to determine whether a patient has a ''definite'' or ''undifferentiated'' connective tissue disease.

Treatment

Treatment largely depends upon individual disease progression and the nature of presenting symptoms.

Antimalarials Antimalarial medications or simply antimalarials are a type of antiparasitic chemical agent, often naturally derived, that can be used to treat or to prevent malaria, in the latter case, most often aiming at two susceptible target groups, young c ...

corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involve ...

, and other drugs may be prescribed, if deemed appropriate by the treating physician.

Prognosis

Most patients will maintain a diagnosis of undifferentiated connective tissue disease. However, about one third of UCTD patients will differentiate to a specific autoimmune disease, like

rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involv ...

or systemic sclerosis. About 12 percent of patients will go into remission. Severe

vitamin D Vitamin D is a group of fat-soluble secosteroids responsible for increasing intestinal absorption of calcium, magnesium, and phosphate, and many other biological effects. In humans, the most important compounds in this group are vitamin D3 (c ...

deficiency has been associated with the progression of UCTD into defined connective tissue diseases. The presence of the

autoantibodies An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies. Pr ...

anti-dsDNA,

anti-Sm Antinuclear antibodies (ANAs, also known as antinuclear factor or ANF) are autoantibodies that bind to contents of the cell nucleus. In normal individuals, the immune system produces antibodies to foreign proteins (antigens) but not to human prote ...

, and anti-cardiolipin has been shown to correlate with the development of

, specifically.

References

External links

{{Medical resources , DiseasesDB = , ICD10 = {{ICD10, M35.9 , ICD9 = {{ICD9, 710.9 , ICDO = , OMIM = , MedlinePlus = , MeSH = , GeneReviewsNBK = , GeneReviewsName = , Orphanet = 90002 Autoimmune diseases Connective tissue diseases Systemic connective tissue disorders