Undifferentiated Connective Tissue Disease – In-Depth | HSS
9 hours ago Abstract. Objective: Undifferentiated connective tissue disease (UCTD) refers to a cluster of systemic disorders characterized by a simple clinical and autoantibody profile. Previously, we had described a series of 91 patients with UCTD who were followed at our unit for a minimum period of one year; here we report the extended followup of these patients. >> Go To The Portal
How is UCTD diagnosed clinically?
At the present time, UCTD is diagnosed clinically by a doctor when the symptoms, lab results and history fit the common patterns doctors see in relation to this disease. It is not based on meeting a checklist of required criteria alone. As doctors develop more specific criteria for UCTD, however,...
What is UCTD and how common is it?
As many as a quarter of all patients seen by rheumatologists have UCTD. Many researchers have been studying people with UCTD to identify serologic profiles (markers in the blood) that may predict who will eventually develop a well-defined connective tissue disease.
What is the pathogenesis of UCTD?
Pathogenesis refers to the origin and development of a disease. The actual cause of UCTD, like many rheumatic diseases, is not well understood. Indeed, there have been no rigorous attempts to define the basic science of UCTD.
What are the signs and symptoms of UCTD (uterine tract infection)?
While most studies note that the majority of patients with UCTD have ANAs, a broad range of immunologic abnormalities can be seen in people with UCTD. These may include: Elevated sedimentation rate (also known as erythrocyte sedimentation rate or ESR), an indicator of inflammation.
Is UCTD a real disease?
This autoimmune disease can share symptoms with other chronic conditions, but it's a distinct condition that is often challenging to diagnose. Maybe it's the word “undifferentiated” that makes undifferentiated connective tissue disease (UCTD) seem a little vague. But it is a real medical condition.
Is UCTD serious?
Overall, patients with UCTD have an excellent prognosis. Although patients with UCTD typically do not have major organ damage or severe symptoms that require long-term immunosuppressive medications, they often suffer from fatigue, depression, and anxiety which can lead to impaired quality of life.
Does UCTD weaken your immune system?
Instead of serving to fight infections such as bacteria and viruses, the body's own immune system attacks itself. In UCTD, autoimmunity may cause the immune system to attack specific parts of the body, resulting in a variety of problems.
Can UCTD affect the brain?
Common symptoms associated with UCTD Problems with the kidneys, liver, lungs or brain are extremely rare.
Can UCTD be cured?
It is important to recognize that there is no cure for UCTD. Therefore, early recognition and treatment of the disease is essential. In addition, because it is a chronic disease, people often require medical therapy for many years.
What is considered a high anti RNP level?
A negative antinuclear ribonucleoprotein (anti-RNP) antibody result is defined as less than 20 U based on enzyme-linked immunoassay (ELISA). A borderline result is defined as 20-25 U. A positive result is defined as more than 26 U.
How serious is connective tissue disease?
Mixed connective tissue disease can lead to serious complications, some of which can be fatal. Complications include: High blood pressure in the lungs (pulmonary hypertension). This condition is a major cause of death in people with mixed connective tissue disease.
Can RNP antibodies disappear?
High titers of Sm and RNP antibodies have been reported in patients with less renal and central nervous system disease, though others have refuted these findings. Sm antibodies may disappear with treatment, while RNP antibodies persist.
Can RNP be false positive?
Although strongly associated with connective tissue diseases, RNP antibodies are not considered a "marker" for any particular disease except in the following situation: when found in isolation (ie, dsDNA antibodies and Sm antibodies are not detectable), a positive result for RNP antibodies is consistent with the ...
Can UCTD cause hair loss?
Other symptoms associated with UCTD include: dry eyes. dry mouth. hair loss.
How do you manage UCTD?
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.
How do you manage undifferentiated connective tissue disease?
Treatment. Treatment for the symptoms of undifferentiated connective tissue (UCTD) disease typically involves the use of nonsteroidal anti-inflammatory drugs (such as naproxen or celecoxib), antimalarial drugs (such as hydroxychloroquine), and/or corticosteroids (such as prednisone ).
What is a UCTD?
Undifferentiated connective tissue disease (UCTD) is a condition in which a patient's symptoms don't quite meet the "criteria" (the markers or indicators doctors use to make a diagnosis) of a well-defined connective tissue disease, such as rheumatoid arthritis (RA), lupus or scleroderma. A person with UCTD may have one or more ...
Why is UCTD contagious?
For unknown reasons, the immune system, which normally fights off invaders such as bacterial and viral infections, may start to think of the body itself as foreign and begin to fight against it. Luckily, in UCTD, the autoimmunity seems to occur at low levels, because there is not much tissue damage seen in patients with UCTD. UCTD is not contagious.
Can UCTD be negative?
The lab tests for these diseases may even be negative (suggesting the disease is not present) in patients with UCTD. The below is a brief overview of UCTD.
Does this patient have undifferentiated connective tissue disease?
Undifferentiated connective tissue disease (UCTD) is a term suggested by LeRoy 30 years ago to denote autoimmune disease that does not meet criteria for established illnesses such as systemic lupus erythematosus, scleroderma, dermatomyositis, Sjogren’s syndrome, vasculitis, or rheumatoid arthritis.
What tests to perform?
The following tests should be considered part of the obligatory evaluation of the patient with presumed autoimmunity. They are ordered at presentation and repeated at intervals predicated on the patient’s course but often at yearly intervals in the stable patient to assess disease activity and pattern.
How should patients with undifferentiated connective tissue disease be managed?
Management of patients with UCTD, similar to patients with SLE, is predicated upon the symptomatic manifestation noted below, or specific finding where treatment has been shown to positively alter outcome. The underlying assumption is that patients will generally have a favorable course.
What happens to patients with undifferentiated connective tissue disease?
One way of looking at this problem, is to think of a universe of people exhibiting some degree of autoimmunity. On the simplest level, an individual might have an autoantibody with no other manifestation of disease. Another patient might have an autoantibody and a single complaint without objective inflammation in an end organ.
How to utilize team care?
For the patient cared for by a primary physician, referral to a rheumatologist will aid in ensuring evaluation is adequate and with interpretation of abnormal results. Some findings on physical examination, i.e. nail fold capillary microscopy will require some experience to ensure proper identification of abnormal loops.
What is the evidence?
Mosca, M, Tani, C, Bombardieri, S. “Undifferentiated connective tissue diseases (UCTD): simplified systemic autoimmune diseases”. Autoimmun Rev. vol. 10. 2011. pp. 256-8.
What is the treatment for UCTD?
Common treatments include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, immunosuppressive agents, calcium channel blockers, and cyclooxygenase-2 inhibitors.
How many antibodies are there for UCTD?
About 80 percent of patients with UCTD have only one auto antibody, usually anti-Ro or anti-RNP. In some cases, patients who are initially diagnosed with Raynaud’s phenomenon alone go on to develop undifferentiated connective tissue disease.
What is undifferentiated connective tissue disease?
Undifferentiated Connective Tissue Disease is found worldwide and affects all races of people although studies suggest that whites are most likely to be affected.
What is the role of deuterium in the cell?
The deuterium interacts with water molecules and creates oxygen at the surface of the cells. Viruses and other pathogens may also be attracted to Ronuv low deuterium water, and would be damaged by the oxygen that is created by the deuterium that is also attracted to it.
Background
At the start of 2019, I had randomly broken out in eczema on my arms, face - which wasn’t responding to treatment ( I’d last had eczema as a child). I was frustrated. I tried allergy tests and at one point the specialist vaguely suggested I should move back to Australia.
Initial treatment and diagnosis
My diagnosis was swift - In this, I seem to be lucky; reading other patient stories, some have waited years for their diagnosis.
Worst experience so far - UCTD and long haul travel
I was apprehensive about a recent holiday to the other side of the world. What will my body do while flying for 24 hours? How painful will my jet lag be? How will my disease cope with 13 hour timezone change?
Coronavirus and UCTD
Coronavirus lockdown and UCTD was scary - given drug combinations I was vulnerable / needed to isolate. I worry about catching this, but hopefully coming off steroids has reduced that risk.
Summary
I expected a quick fix and that didn’t happen. I’ve had to change my expectations around the disease and accept that there isn’t a sure-fire cure.
What are the symptoms of UCTD?
Symptoms of UCTD vary, but more common symptoms include Raynaud phenomenon, arthritis, joint pain, fever, and symptoms involving the mucous membranes and skin such as dryness in the eyes and mouth, ulcers in the mouth, sensitivity to sunlight (photosensitivity), or hair loss. [1] .
What is a UCTD?
Listen. Undifferentiated connective tissue disease (UCTD) is type of autoimmune disease, which means that the immune system malfunctions and mistakenly "attacks" a person's own healthy tissues. However, like many autoimmune diseases, the reason a person develops UCTD is not known. Researchers suspect that both genes and environmental triggers ...
What is undifferentiated connective tissue disease?
Undifferentiated connective tissue disease (UCTD) is an autoimmune disease that can affect several systems in the body. Connective tissue disease (CTD) is classified as undifferentiated CTD when signs and symptoms are consistent with a CTD, but do not fulfill the diagnostic or classification criteria for one of the previously defined CTDs (for example, rheumatoid arthritis or lupus ). [1] Symptoms of UCTD vary, but more common symptoms include Raynaud phenomenon, arthritis, joint pain, fever, and symptoms involving the mucous membranes and skin such as dryness in the eyes and mouth, ulcers in the mouth, sensitivity to sunlight (photosensitivity), or hair loss. [1] Various other symptoms may be present and may additionally involve the lungs, heart, muscles, or nervous system. [1]
What is the autoimmune registry?
The Autoimmune Registry supports research for Undifferentiated connective tissue disease by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
What is UCTD in 2020?
June 9, 2020. Undifferentiated connective tissue disease (UCTD) is a term given to an autoimmune disease affecting the connective tissue. Autoimmune diseases occur when your body’s defense system, the immune system, is too active.
What is the difference between UCTD and lupus?
The difference between UCTDs and connective tissue diseases (like lupus) Being diagnosed with UCTD does not necessarily mean you will develop CTD. 3 Out of every 10 people diagnosed with UCTD, 2 will develop CTD. 3 Three people will recover completely, and their symptoms disappear. 3 The remaining 5 will live with some mild symptoms of UCTD. 3.
How do you know if you have Raynaud's disease?
Rash, especially on the face. Alopecia (loss of hair) Dry mouth and eyes. Mouth ulcers. Low-grade fever. Photosensitivity – easily sunburned and sensitive to light. Raynaud’s phenomenon – lack of blood to your hands and feet which makes them look blue when you get cold.
Can a doctor monitor a UCTD?
If you have UCTD, talk to your doctor if you are pregnant or thinking of getting pregnant. CTDs like lupus make pregnancy risky. 6 Since some UCTDs turn into CTDs, doctors may want to monitor you. Your doctor may monitor your thyroid. The thyroid is an organ in the neck.
Do UCTDs cause more damage than UCTDs?
They usually cause more damage than UCTDs. 3 If you are diagnosed with UCTD, you likely share some symptoms of a CTD but maybe not the trademark symptoms. 1. UCTDs and CTDs are very different in terms of treatment and outcome. 1,3 Just because you have UCTD does not mean you will end up with a CTD like lupus. 1,3.
Terminology
Symptoms
- The exact cause is not well understood, however, UCTD is believed to be triggered by environmental factors (such as exposure to certain bacteria or viruses) and/or genetics (biological characteristics inherited from a person's parents). Like other, well-defined connective tissue diseases, UCTD is thought to be a systemic autoimmune disorder. For un...
Diagnosis
Pathophysiology
Research
- The most common presenting manifestations of this syndrome are arthralgia/myalgia, arthritis, Raynauds phenomenon, sicca syndrome and fatigue. Most authors suggest the patient have a positive ANA to be included within this group. Less commonly encountered findings include urticaria, photosensitivity, mucosal ulcerations, serositis, thyroiditis, cytopenias, and rash.
Contraindications
- A literature has developed linking UCTD with idiopathic nonspecific interstitial pneumonia (NSIP). While only 20% of cohorts of UCTD will have pulmonary involvement, a high percentage of patients with idiopathic nonspecific interstitial pneumonia will have UCTD. Recent classification of interstitial lung disease by the American Thoracic Society has separated NSIP from usual interstitial pneumonitis (UIP). The former is manifest with a uniform patt…
Evolution
- Since Raynauds is a common feature of both UCTD and defined rheumatic disease (SLE, scleroderma, dermatomyositis and rheumatoid arthritis (RA) there have been efforts to define abnormalities morphologically as well as biochemically in the vasculature of these patients. In one study, scleroderma type loops have been found in a small percentage of UCTD patients (approximately 14%) suggesting increased risk for progression to sclero…
Clinical significance
- Vitamin D and its influence on the immune system has been a topic of increasing interest this past decade. A higher incidence of autoimmunity in patients deficient in vitamin D has been suggested. Patients with UCTD with low vitamin D levels have been reported to have a higher rate of progression to defined connective tissue disease. Recent data su...
Cause
- Anti-histone antibody should be ordered when drug induced lupus is suspected. Chest X-ray (CXR) should be obtained in all patients to exclude parenchymal disease, i.e., interstitial fibrosis, as well as to exclude adenopathy, which may denote sarcoidosis or lymphoma presenting as a paraneoplastic syndrome.
Toxicity
- Anti-centromere and Scl-70 antibodies these are highly specific for limited (CREST syndrome) and generalized scleroderma respectively in symptomatic patients. While both may be seen in patients without evidence of scleroderma or in the case of anti-centromere antibody of primary biliary cirrhosis, its presence in association with Raynauds suggests evolution toward those entities. Isolated anti-Scl-70 is uncommonly observed in the otherwis…
Issues
- Antibodies to myositis antigens these are associated with polymyositis and dermatomyositis. Anti-Jo-1 associates with dermatomyositis, digital dermatitis (mechanics hands), and interstitial lung disease.
Healthcare
- Angiotensin converting enzyme seen in 50% of patients with sarcoidosis, a condition which mimics connective tissue disease both with constitutional symptoms, joint pain and often positive ANA and rheumatoid factor.
Prognosis
- Cytopenias while leucopenia and thrombocytopenia may be seen in UCTD, their presence should bias the observer toward SLE.
Treatment
- Imaging ask yourself: which imaging studies should I order (discuss them in the order of the information they provide to the physician)? Does the imaging modality matter? How do I interpret imaging results? How often should I order imaging tests. What are the next steps?
Adverse effects
- Lymph node biopsies, excisional or fine needle aspirate, when lymphadenopathy is detected and considered clinically significant (rule out lymphoma, sarcoidosis).
Management
- Management of patients with UCTD, similar to patients with SLE, is predicated upon the symptomatic manifestation noted below, or specific finding where treatment has been shown to positively alter outcome. The underlying assumption is that patients will generally have a favorable course. As previously noted, 70% of patients with UCTD will persist with presenting manifestations and not progress to a defined rheumatic disease. Early in t…
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