Lupus In 'Overlap' With Other Connective Tissue Diseases

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Adminஐﻬ:
Mixed connective tissue disease
Overview
Mixed connective tissue disease (MCTD) is a rare autoimmune disorder that causes signs and symptoms of other connective tissue diseases. People with mixed connective tissue disease experience features ஐﻬof three other diseases — lupus, scleroderma and polymyositis. For this reason, mixed connective tissue disease is sometimes referred to as an overlap disease.

Signs and symptoms of these three other diseases usually don't appear all at once. This makes diagnosing mixed connective tissue disease somewhat complicated. Often people with mixed connective tissue disease are first diagnosed with lupus. As the disease progresses and other signs and symptoms become apparent, the diagnosis is corrected.

Mixed connective tissue disease occurs most often in women and is usually diagnosed in young adults in their 20s and 30s. Children have also been diagnosed with mixed connective tissue disease.

Mixed connective tissue disease is somewhat of a controversial term among arthritis specialists (rheumatologists). Some question whether mixed connective tissue disease is its own specific disease or whether it's a precursor to another connective tissue disease.

Signs and symptoms
Mixed connective tissue disease doesn't have a unique set of signs and symptoms. Instead, people with ஐﻬmixed connective tissue disease usually have signs and symptoms of lupus, scleroderma and polymyositis, including:

Fatigue
Muscle weakness
Joint pain
Joint swelling
Swollen fingers
Mild fever
Raynaud's phenomenon — blood vessel spasms that interrupt blood flow to the fingers, toes, ears and nose
Causes
Doctors don't know what causes mixed connective tissue disease. The disease is part of a larger group of diseases known as autoimmune disorders. When you have an autoimmune disorder, your immune system — the part of your body responsible for fighting off disease — mistakes normal, healthy cells for intruders. As a result, healthy tissue in your body is damaged, causing signs and symptoms of disease.

It isn't clear what causes your immune system to attack your body. Doctors believe a complex mix of viruses, chemicals and genetic factors may be at play.

Risk factors
Doctors don't know what puts you at risk of mixed connective tissue disease. Some research shows the disease may occur more frequently in people with a family history of connective tissue diseases. Other findings show an increased risk in people exposed to certain chemicals, including vinyl chloride and silica. More research is needed to confirm these findings.

When to seek medical advice
Signs and symptoms of mixed connective tissue disease usually begin mildly and may not prompt you to seek medical attention. But if signs and symptoms become bothersome or interfere with your daily routine, make an appointment with your doctor.

Also see your doctor if you've been diagnosed with lupus or another connective tissue disease and you begin developing new signs and symptoms.

Screening and diagnosis
Your doctor may suspect mixed connective tissue disease based on your signs and symptoms. He or she will conduct a physical exam to look for signs such as swollen hands and painful, swollen joints.

A blood test determines whether you have a certain antibody in your blood that indicates mixed connective tissue disease. The presence of this specific antibody — called U1-RNP — can confirm your doctor's suspicions.

Mixed connective tissue disease usually develops slowly, making it difficult to diagnose. As your signs and symptoms evolve over time — sometimes many years — your diagnosis may change. Many people are first diagnosed with lupus and later re-diagnosed with mixed connective tissue disease. ஐﻬOthers begin with a diagnosis of mixed connective tissue disease only to later find they have lupus or another connective tissue disorder.

Complications
Mixed connective tissue disease and its treatment can lead to serious complications, including:

Pulmonary hypertension. High blood pressure affecting the arteries in your lungs (pulmonary hypertension) is the most common cause of death in people with mixed connective tissue disease. You might experience difficulty breathing or chest pain if you have pulmonary hypertension. People with mixed connective tissue disease usually need to take medications to control pulmonary hypertension.
Heart disease. People with mixed connective tissue disease are at risk of developing heart conditions, including enlargement of parts of the heart and inflammation around the heart (pericarditis). ஐﻬYour doctor may routinely monitor your heart with an electrocardiogram.
Side effects of long-term steroid use. Steroids are commonly used to manage the signs and symptoms of mixed connective tissue disease. While these medications are effective, they don't come without risks. If you take steroids, your doctor will likely monitor you for adverse effects, such as bone loss due to osteoporosis or avascular necrosis, muscle weakness and infection.
Pregnancy complications. Women with mixed connective tissue disease may experience flares during pregnancy. Babies born to women with mixed connective tissue disease are at risk of being born with a low birth weight. If you're planning to become pregnant, talk with your doctor about this risk.
Treatment
No cure exists for mixed connective tissue disease, although treatments can help manage the signs and symptoms of the disease. Your treatment may vary from another person's because your signs and symptoms may be different.

While no standard treatment exists, the most common treatment for mixed connective tissue disease is corticosteroids, such as prednisone.

People with mild forms of mixed connective tissue disease may not need any treatment. You may require treatment only during flares or you may require constant medication. Work with your doctor to ensure that your signs and symptoms are adequately controlled.

Coping skills
Living with a chronic disease that has no cure requires effective coping skills. Consider trying to:

Find out as much as you can about mixed connective tissue disease. Learn as much as you can about the disease. Ask your doctor and other health care team members for assistance in locating reliable resources. The more you know about the disease, the easier it is to understand what's happening to your body.
Take care of yourself. Control your health as best you can. Eat a balanced diet with plenty of fruits and vegetables. Get exercise on days you feel up to it. Keeping your body healthy makes you better able to deal with the daily stress of living with a chronic illness. And it better prepares you ஐﻬto cope with your next flare.
Seek support from others. Social support helps you cope with the stress of mixed connective tissue disease. Ask your doctor about support groups in your area for people with chronic illnesses. Go online to connect with other people living with mixed connective tissue disease.

Adminஐﻬ:
Mixed connective tissue disease

Overview
Mixed connective tissue disease (MCTD) is a rare autoimmune disorder that causes signs and symptoms of other connective tissue diseases. People with mixed connective tissue disease experience features of three other diseases — lupus, scleroderma and polymyositis. For this reason, mixed connective tissue disease is sometimes referred to as an overlap disease.

Signs and symptoms of these three other diseases usually don't appear all at once. This makes diagnosing mixed connective tissue disease somewhat complicated. Often people with mixed connective tissue disease are ஐﻬfirst diagnosed with lupus. As the disease progresses and other signs and symptoms become apparent, the diagnosis is corrected.

Mixed connective tissue disease occurs most often in women and is usually diagnosed in young adults in their 20s and 30s. Children have also been diagnosed with mixed connective tissue disease.

Mixed connective tissue disease is somewhat of a controversial term among arthritis specialists (rheumatologists). Some question whether mixed connective tissue disease is its own specific disease or whether it's a precursor to another connective tissue disease.

Signs and symptoms
Mixed connective tissue disease doesn't have a unique set of signs and symptoms. Instead, people with mixed connective tissue disease usually have signs and symptoms of lupus, scleroderma and polymyositis, including:

Fatigue
Muscle weakness
Joint pain
Joint swelling
Swollen fingers
Mild fever
Raynaud's phenomenon — blood vessel spasms that interrupt blood flow to the fingers, toes, ears and nose
Causes
Doctors don't know what causes mixed connective tissue disease. The disease is part of a larger group of diseases known as autoimmune disorders. When you have an autoimmune disorder, your immune system — the part of your body responsible for fighting off disease — mistakes normal, healthy cells for intruders. As a result, healthy tissue in your body is damaged, causing signs and symptoms of disease.

It isn't clear what causes your immune system to attack your body. Doctors believe a complex mix of viruses, chemicals and genetic factors may be at play.

Risk factors
Doctors don't know what puts you at risk of mixed connective tissue disease. Some research shows the disease may occur more frequently in people with a family history of connective tissue diseases.ஐﻬ Other findings show an increased risk in people exposed to certain chemicals, including vinyl chloride and silica. More research is needed to confirm these findings.

When to seek medical advice
Signs and symptoms of mixed connective tissue disease usually begin mildly and may not prompt you to seek medical attention. But if signs and symptoms become bothersome or interfere with your daily routine, make an appointment with your doctor.

Also see your doctor if you've been diagnosed with lupus or another connective tissue disease and you begin developing new signs and symptoms.

Screening and diagnosis
Your doctor may suspect mixed connective tissue disease based on your signs and symptoms. He or she will conduct a physical exam to look for signs such as swollen hands and painful, swollen joints.

A blood test determines whether you have a certain antibody in your blood that indicates mixed connective tissue disease. The presence of this specific antibody — called U1-RNP — can confirm your doctor's suspicions.

Mixed connective tissue disease usually develops slowly, making it difficult to diagnose. As your signs and symptoms evolve over time — sometimes many years — your diagnosis may change. Many people are first diagnosed with lupus and later re-diagnosed with mixed connective tissue ஐﻬdisease. Others begin with a diagnosis of mixed connective tissue disease only to later find they have lupus or another connective tissue disorder.

Complications
Mixed connective tissue disease and its treatment can lead to serious complications, including:

Pulmonary hypertension. High blood pressure affecting the arteries in your lungs (pulmonary hypertension) is the most common cause of death in people with mixed connective tissue disease. You might experience difficulty breathing or chest pain if you have pulmonary hypertension. People with mixed connective tissue disease usually need to take medications to control pulmonary hypertension.

Heart disease. People with mixed connective tissue disease are at risk of developing heart conditions, including enlargement of parts of the heart and inflammation around the heart (pericarditis). Your doctor may routinely monitor your heart with an electrocardiogram.
Side effects of long-term steroid use.
 Steroids are commonly used to manage the signs and symptoms of mixed connective tissue disease. While these medications are effective, they don't come without risks. If you take steroids, your doctor will likely monitor you for adverse effects, such as bone loss due to osteoporosis or avascular necrosis, muscle weakness and infection.
Pregnancy ஐﻬcomplications. Women with mixed connective tissue disease may experience flares during pregnancy. Babies born to women with mixed connective tissue disease are at risk of being born with a low birth weight. If you're planning to become pregnant, talk with your doctor about this risk.


Treatment
No cure exists for mixed connective tissue disease, although treatments can help manage the signs and symptoms of the disease. Your treatment may vary from another person's because your signs and symptoms may be different.

While no standard treatment exists, the most common treatment for mixed connective tissue disease is corticosteroids, such as prednisone.

People with mild forms of mixed connective tissue disease may not need any treatment. You may require treatment only during flares or you may require constant medication. Work with your doctor to ensure that your signs and symptoms are adequately controlled.

Coping skills
Living with a chronic disease that has no cure requires effective coping skills. Consider trying to:

Find out as much as you can about mixed connective tissue disease. Learn as much as you can about the disease. Ask your doctor and other health care team members for assistance in locating reliable resources. The more you know about the disease, the easier it is to understand what's happening to your body.
Take care of yourself. Control your health as best you can. Eat a balanced diet with plenty of fruits and ஐﻬvegetables. Get exercise on days you feel up to it. Keeping your body healthy makes you better able to deal with the daily stress of living with a chronic illness. And it better prepares you to cope with your next flare.
Seek support from others. Social support helps you cope with the stress of mixed connective tissue disease. Ask your doctor about support groups in your area for people with chronic illnesses. Go online to connect with other people living with mixed connective tissue disease.


www.LupusMCTD.com

Adminஐﻬ:
Systemic Sclerosis
A chronic disease of unknown cause, characterized by diffuse fibrosis; degenerative changes; and vascular abnormalities in the skin (scleroderma), articular structures, and internal organs (especially the esophagus, GI tract, lung, heart, and kidney).

Systemic sclerosis is about four times more common in women than men and is comparatively rare in children.

Localized forms of scleroderma occur as circumscribed patches (morphea) or linear sclerosis of the integument and immediately subjacent tissues without systemic involvement. Mixed connective tissue disease (MCTD--see below) ஐﻬcombines features of scleroderma (eg, Raynaud's phenomenon, esophageal dysfunction) with clinical and serologic features of SLE, polymyositis, or RA. Patients with MCTD have extremely high titers of a serum antibody that reacts with nuclear ribonucleoprotein.

Symptoms, Signs, and Diagnosis
Systemic sclerosis varies in severity and progression, ranging from generalized cutaneous thickening (systemic sclerosis with diffuse scleroderma), which may cause rapidly progressive and often fatal visceral involvement, to a form distinguished by restricted skin involvement (often just the fingers and face) and slow progression, often several decades, before full manifestation of characteristic internal involvement. This latter form is termed limited cutaneous scleroderma or CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasia). In addition, overlap syndromes exist; eg, sclerodermatomyositis (tight skin and muscle weakness indistinguishable from polymyositis); MCTD; and a musculoskeletal syndrome chemically induced by certain systemic poisons, as occurred in the ingested toxic oil syndrome in Madrid in 1981, which affected about 20,000 people.ஐﻬ A syndrome of incapacitating myalgias and eosinophilia was associated with the ingestion of L-tryptophan, although the exact cause is unknown, and the toxicity probably resulted from a contaminant.

The most common initial complaints in systemic sclerosis are Raynaud's phenomenon and insidious swelling of the acral portions of the extremities, with gradual thickening of the skin of the fingers. Polyarthralgia is also prominent. GI disturbances (eg, heartburn, dysphagia) or respiratory complaints (eg, dyspnea) are occasionally the first manifestations of the disease.

Skin: Induration is symmetric and may be confined to the fingers (sclerodactyly) and distal portions of the upper extremities, or it may affect most or all of the body. As the disease progresses, the skin becomes taut, shiny, and hyperpigmented; the face becomes masklike; and telangiectases appear on the fingers, chest, face, lips, and tongue. Subcutaneous calcifications may develop (calcinosis circumscripta), usually on the fingertips (pulps) and over bony eminences.ஐﻬ Capillary microscopy of the nail folds reveals dilated capillary loops mixed with areas of loss of the microvascular loops normally visible at that site. Biopsy of indurated skin shows an increase in compact collagen fibers in the reticular dermis, epidermal thinning, loss of rete pegs, and atrophy of dermal appendages. There may be variably large accumulations of T lymphocytes in the dermis and subcutis, which also may be the site of extensive fibrosis.

Musculoskeletal system: Friction rubs may develop over the joints (particularly the knees), tendon sheaths (tendinitis), and large bursae because of fibrin deposition on synovial surfaces. Flexion contractures of the fingers, wrists, and elbows result from fibrosis of the synovial membrane, periarticular soft tissues, and skin. Trophic ulcers are common, especially on the fingertips, overlying the finger joints, or over calcinotic nodules.

GI tract: Esophageal dysfunction is the most frequent visceral disturbance and eventually occurs in most patients. Dysphagia (manifested by various abnormal swallowing sensations) is initially caused by impaired esophageal motility but later can result from gastroesophageal reflux disease and secondary stricture formation. Acid reflux resulting from lower esophageal sphincter incompetence, and peptic esophagitis with possible ulceration and stricture are common. Barrett's esophagus occurs in 1/3 of patients with scleroderma; these patients have an increased risk of complications (eg, stricture, adenocarcinoma). Hypomotility of the small intestine may be ஐﻬassociated with malabsorption resulting from anaerobic bacterial overgrowth. Pneumatosis cystoides intestinalis may follow degeneration of the muscularis mucosa and entry of air into the submucosa of the intestinal wall. Characteristic large-mouthed sacculations can develop in the colon and ileum because of atrophy of the smooth muscle of these segments. Biliary cirrhosis may be associated with the CREST syndrome.

Cardiorespiratory system: Lung fibrosis causes early impairment in gas exchange leading to exertional dyspnea. Pleurisy and pericarditis with effusion may occur. Lung involvement generally progresses indolently, with substantial individual variability. Pulmonary hypertension may result from long-standing interstitial and peribronchial fibrosis or intimal hyperplasia of small pulmonary arteries; the latter is associated with the CREST syndrome. Cardiac arrhythmias,ஐﻬ conduction disturbances, and other ECG abnormalities are common. Ambulatory ECG in patients with cardiac or pulmonary involvement revealed ventricular ectopy in 67%; the finding was strongly correlated with sudden death. Heart failure may develop because of pulmonary hypertension and secondary cor pulmonale or because of diffuse fibrous replacement of cardiac muscle. Heart failure tends to be chronic and difficult to treat.

Renal system: Severe renal disease may occur as a consequence of intimal hyperplasia of interlobular and arcuate arteries and usually is heralded by the abrupt onset of accelerated or malignant hypertension. If untreated, renal insufficiency rapidly progresses, becomes irreversible, and is lethal within a few months. However, modern aggressive antihypertensive therapy has made survival for >= 2 yr common, although not all patients respond and some progress to renal failure despite good BP control (see Treatment, below).

Laboratory Findings
Full-blown systemic sclerosis is readily diagnosed on clinical grounds. The nonspecific antibody and HLA typing are largely of research interest. Rheumatoid factor tests are positive in 33% of systemic sclerosis patients, and serum ANA are present in >= 90%. The ANA often show an antinucleolar pattern. An antibody that reacts with centromeric protein (anticentromere antibody) occurs in the serum of a high proportion of patients with the CREST syndrome. SCL-70 antigen (topoisomerase I) is a DNA-binding protein sensitive to nucleases. Patients with diffuse scleroderma are more likely to have anti-SCL-70 antibodies. Anti-SCL-70 antibodies were associated with peripheral ஐﻬvascular disease and pulmonary interstitial fibrosis but were not predictive of cardiac or renal involvement or survival. Analysis of various HLA types and scleroderma shows a significant correlation only between systemic sclerosis and HLA-DR5 and an increased frequency of HLA-DR1 in patients with the CREST syndrome.

Prognosis
The course is variable and unpredictable and often progresses slowly. Most patients eventually show evidence of visceral involvement. Prognosis is poor if cardiac, pulmonary, or renal manifestations are present early. However, the disease may remain limited and nonprogressive for long periods in patients with the CREST syndrome; other visceral changes (eg, pulmonary hypertension caused by vascular disease of the lung, a peculiar form of biliary cirrhosis) eventually develop, but the course of this form of systemic sclerosis often is remarkably benign.

Treatment
No drug has significantly influenced the natural history of systemic sclerosis, but various drugs are of value in treating specific symptoms or organ systems. Corticosteroids may be helpful for disabling myositis, synovitis, or MCTD. Prolonged oral administration (> 1.5 yr) of penicillamine (0.5 to 1.0 g/day) can reduce skin thickening and may delay the rate of new visceral involvement. The drug is usually started at 250 mg/day and increased at several monthly intervals to improve tolerance. Various immunosuppressive drugs, including methotrexate, have been anecdotally useful in some cases of systemic sclerosis; a double-blind study of 65 patients revealed no benefit after 3 yr of immunosuppressive treatment with chlorambucil. Nifedipine 20 mg tid or as tolerated may help control Raynaud's phenomenon. Reflux esophagitis is relieved byஐﻬ frequent small feedings, antacids, and H2 blockers (eg, cimetidine 300 mg qid 30 min before meals and at bedtime) or with proton pump inhibitors and by having the patient sleep with the head of the bed elevated. Esophageal strictures may require periodic dilation; successful correction of gastroesophageal reflux by gastroplasty has been reported. Tetracycline 1 g/day po or another broad-spectrum antibiotic suppresses overgrowth of intestinal flora and may alleviate intestinal malabsorption symptoms caused by bacterial colonization of dilated bowel loops. Physiotherapy may help preserve muscle strength but is ineffective in preventing joint contractures.

For renal disease, ACE inhibitors are the drugs of choice. Other vasodilators (eg, minoxidil) also have been used with some success. All of these drugs effectively control hypertension and help preserve renal function. When end-stage renal disease is unpreventable, dialysis and transplantation can be used, although the mortality rate remains high.

Adminஐﻬ:
What is Mixed Connective Tissue Disease (MCTD)?

A few decades ago, scientists discovered that patients with a connective tissue disease had symptoms of both lupus, scleroderma, myositis, etc. These diseases are called ‘overlap-syndromes’. In 1969 Sharp and his co-workers found out that there is one specific type of overlap-syndromeஐﻬ with symptoms of lupus, scleroderma, myositis and rheumatoid arthritis, together with a large quantity of antibodies against one specific antigen, namely U1RNP. They believed it to be a distinct disease entity and called it MCTD. Meanwhile, it is known that Sjogren’s Syndrome is very common in MCTD.
It is still a matter of debate whether or not this disorder is diverse from the other overlap-syndromes. In general, however, this disorder is considered a distinct clinical entity.
 
Most important symptoms
Raynaud’s phenomenon
Is basically always present in the early phase of the disease, mostly without other symptoms at the level of the fingers, unless scleroderma is also present at the onset of the disease. 
 
Swollen fingers
Mostly all the fingers are swollen in the overall length and become ‘sausage-like’. Sometimes this is only temporary, but occasionally it evolves into sclerodactyly (thin fingers with hard skin and limited mobility). 

Arthritis
In the early phase there is painful swelling of the joints of the hands and feet like in rheumatoid arthritis. ஐﻬDamage to the cartilage or bone, however, is rare. As such, malformations do not occur or only seldom and the function of the joints remains intact. This kind of arthritis is comparable to the arthritis seen in lupus. 
 
Muscle inflammation
In 10 to 20% of the cases, patients develop a real form of myositis, muscle inflammation (see polymyositis). Two out of three patients suffer from significant muscle pain (no weakening or paralysis), without demonstrable abnormalities in the laboratory test, electromyography or biopsy. Patients complain mostly of pain at the level of the large muscle groups of the shoulder girdle and the upper arms.

Lungs
The lungs may show the same abnormalities and problems as in scleroderma. Reduced lung volume is common, sometimes reduced absorption of oxygen and rarely overpressure in the lung vessels with fatal outcome occurs.

Oesophagus
The same complaints as in scleroderma may occur. 
 
Heart
Inflammation of the heart sac or pericardium (pericarditis) may be acute. Unlike in polymyositis, inflammation of the heart muscle (myocarditis), which may cause heart failure or arrhythmia, occurs rarely. These complications are very serious and may be life threatening. 
 
Neurological damage
Meningitis, psychological abnormalities due to brain damage, damage to the spinal marrow or facial nerves have been described. These symptoms occur often in Sjogren’s Syndrome, which often occurs in MCTD.
 
Renal involvement
Renal involvement is very rare and can be similar to the damage caused in scleroderma (especially damage to the renal blood vessels) as well as to the damage caused in lupus lupus (damage to the renal filtering units).

Skin and mucous membranes
The skin may show symptoms of scleroderma as well as of lupus. The mucous membranes (mouth, vagina) and the eyes may be dry due to the Sjogren’s Syndrome .
 
Who can get MCTD and is it hereditary?
MCTD is a rare disease. In most cases the age of onset lies between 20 and 50 and 8 to 9 out of 10 patients are female. MCTD is not hereditary. There can be a difference in the disease’s genetic susceptibility: the same susceptibility often seen in lupus, rheumatoid arthritis or Sjogren’s Syndrome, can occur in combination.
External factors may also play a role. Some cases of MCTD have been described after the patients had worked professionally with PVC (polyvinylchloride).

  Evolution
Since MCTD overlaps (‘mixes’) different connective tissue disorders, there are many different types, and consequently, many prognoses. Complaints and symptoms depend on the organs involved, the degree ofஐﻬ inflammation and the general degree of disease activity. In general the prognosis is favourable if the disease is adequately treated. Life expectancy is comparable to the life expectancy in lupus, i.e. favourable with a few exceptions.

Diagnosis
The diagnosis is based on complaints, symptoms and organ involvement and on the presence of anti-U1RNP antibodies in high titre (concentration). It is the only connective tissue disease for which one specific type of antibody is necessary to make a diagnosis (at least in most centres). If the doctor suspects the diagnosis of MCTD, some additional tests like lung function tests, heart check-up, examination of the kidneys and blood pressure need to be carried out. In case of muscle complaints or nerve pains an electromyography is necessary. The results of these examinations may also contribute to a diagnosis.

Treatment
Although treatments with a low dose of corticosteroids have been successful, there is no standard procedure for MCTD. The treatment is based on the type and degree of organ involvement, and can be a treatment for lupus or for scleroderma. The treatment has to be individualised for each patient.
 
MCTD and pregnancy
Surveys on the subject are quite different. The influence of pregnancy on MCTD or vice versa can be compared to pregnancy in lupus.
 
Overlapping syndromes
Besides MCTD there exist also other overlap syndromes which are less clearly definable or which do not have anti-U1RNP antibodies. As in MCTD, treatment is based on the type and degree of organ involvement and it is individualised for each patient. The evolution and prognosis are also comparable to MCTD, except that there may be more individual dissimilarities among patients.
 
Undefined Connective Tissue Disease (UCTD)
 In the early phase of a connective tissue disease and especially at the onset of an overlap syndrome or MCTD, the symptoms can be very limited and unusual. Although the onset of a connective tissue disease can be certain in such cases, it is impossible to define the type of disease with certainty. ஐﻬThat is why the term UCTD (= undefined connective tissue disease) is used. Mostly Raynaud’s phenomenon, joint pains, sometimes arthritis and muscle pains are present. The evolution is very diverse: some patients remain in this phase while others evolve quickly into a real form of lupus or another type of connective tissue disease. Again, treatment has to be individualised according to each patient.

 


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Adminஐﻬ:
MCTD vs. UCTD
(Mixed Connective Tissue Disease vs. Undifferentiated Connective Tissue Disease)
Medical Author: William C. Shiel Jr., MD, FACP, FACR

I note that some viewers have been requesting some clarification of the meaning of the terms "mixed connective tissue disease" (MCTD) and "undifferentiated connective tissue disease" (UCTD).

Connective tissue diseases are a special group of rheumatic diseases (diseases that feature abnormalities of the muscles and/or joints) that can be associated with arthritis. The cause(s) for the connective tissue diseases is (are) unknown.ஐﻬ They are characterized as a group by the presence of spontaneous over-activity of the body's immune (defense) system. This over-activity results in the production of unusual antibodies that are found in the blood. The antibodies themselves may or may not cause any problems in patients with connective tissues diseases, but they are commonly found in the blood as an characteristic feature.

The connective tissues are the structural portions of our body that essentially hold the cells of the body together. These tissues form a framework, or matrix, for the body. The connective tissues are composed of two major structural protein molecules, collagen and elastin. There are many different types of collagen protein that vary in amount in each of the body's tissues. Elastin has the capability of stretching and returning to its original length - like a spring or rubber band.ஐﻬ Elastin is the major component of ligaments (tissues that attach bone to bone) and skin. In patients with connective tissue diseases, it is common for collagen and elastin to become injured by inflammation. Diseases in which inflammation of collagen tends to occur are also referred to as collagen diseases.

The classic connective tissue diseases include systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymyositis, and dermatomyositis. Each of these diseases affects people in a characteristic way and causes typical findings that doctors can recognize during an examination. Each also has characteristic blood test abnormalities and abnormal antibody patterns. For example, systemic lupus erythematosus has dsDNA antibodies, while scleroderma has Sc170 antibodies. Additionally, each of these diseases can evolve either slowly or rapidly from very subtle abnormalities before demonstrating the classic features that help in the diagnosis.

When these conditions have not developed the classic features of a particular disease, doctors will often refer to the condition as "undifferentiated connective tissue disease," or UCTD. This designation implies that the characteristic features that are used to define the classic connective tissue diseases are not present, but that some symptoms or signs of a connective tissue disease exist.ஐﻬ For example, a person may have a special antibody in the blood, such as antinuclear antibody and muscle pains, but no other definable features of a classic connective tissue disease. Individuals with undifferentiated connective tissue disease may never develop a fully definable condition or they may eventually develop a classic connective tissue disease.

Mixed connective tissue disease, which was first described in 1972, is "classically" considered as an "overlap," or mix, of three specific connective tissue diseases; systemic lupus erythematosus, scleroderma, and polymyositis. Patients with this pattern of illness (that is, with MCTD) have features of each of these three diseases. They also typically have very high quantities of antinuclear antibodies (ANAs) and antibodies to ribonucleoprotein (anti-RNP) detectable in their blood. The symptoms of many of these patients eventually evolve to become dominated by features of one of the three component illnesses, most commonly the scleroderma features.

It is now known, however, that overlap syndromes can involve any combination of the connective tissue diseases. Therefore, for example, patients can have a combination of rheumatoid arthritis and systemic lupus erythematosus (hence, the coined name "rhupus"). Accordingly, today, true mixed connective tissue disease is diagnosed when patients demonstrate the clinical features ஐﻬ(exam findings) of overlap illnesses. These patients also have high amounts of ANA and anti-RNP without having such other antibodies as the dsDNA antibodies of systemic lupus erythematosus and the Scl70 antibodies of scleroderma.

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