JOINT SYNDROME

A Differential Diagnosis of Joint Syndrome between Scleroderma , Dermatomyositis and

Systemic Lupus Erythematosus by

Daniel Schwartz David (India) Group : 44

SCLERODERMA

• Scleroderma is a chronic systemic autoimmune disease (primarily of the skin) characterized by fibrosis (or hardening), vascular alterations, and autoantibodies.

DERMATOMYOSITIS

It is an acquired myopathy disease which affects a person's muscles that is characterized by both a distinctive skin rash and inflammation.

SYSTEMIC LUPUS ERYTHEMATOSUS

• Systemic Lupus Erythematosus is an autoimmune disease in which the immune system produces antibodies to cells within the body leading to widespread inflammation and tissue damage.

AGE AND SEX

• SCLERODERMA : 30 – 50 years female slightly more vulnerable

• DERMATOMYOSITIS : Adult : 30-50 years • Juvenile : 5-10 years female preponderance

• SLE : 15 – 35 YEARS F : M :: 9 : 1

SCLERODERMA

• ARTHRALGIA• CARPAL TUNNEL SYNDROME • ACROOSTEOLYSIS• SYNOVITIS & TENDON RUB• JOINT SPACE NARROWING & SUBLUXATION• LOSS IN RANGE OF MOTIONS AND FLEXION

CONTRACTURE

SYSTEMIC LUPUS ERYTHEMATOSUS

• Hands are stiff and painful moslty in the morning and lasts for 1 to 2 hours

• INTERMITTENT pain , swelling and effusion in wrists , hands and knees

• JACCOUD’S syndrome : Non- erosive deforming disease , fingers deviate toward the little finger

Jaccoud’s Arthritis

It is “reversible”. 1) subluxation of metacarpophalangeal joints, 2)“swan-neck” 3) Boutonniere deformities 4) “Z” deformity of thumb .

• AVASCULAR NECROSIS OF BONE : In this condition part of the bone loses its blood supply and dies causing necrosis (death of part of the bone) in that area. This then causes severe pain in the joint involved and later collapse of that joint. The most common site is the hip, and if this does occur it usually requires a total joint replacement.

DERMATOMYOSITIS

• . Joint manifestations include polyarthralgia or polyarthritis, often with swelling, effusions, and other characteristics of nondeforming arthritis, which occur in about 30% of patients. However, joint manifestations tend to be mild. They occur more often in a subset with Jo-1 or other antisynthetase antibodies.

X-Ray - Dermatomyositis

Lab Diagnosis

• SYSTEMIC LUPUS ERYTHEMATOSUS ARTHROCENTESIS• Effusions can be inflammatory or noninflammatory. Cell

count will reveal less than 25% polymorphonuclear neutrophils (PMN) in noninflammatory effusions or more than 50% in inflammatory effusions. Viscosity will be high in noninflammatory effusions and low in inflammatory effusions. Gross appearance will be straw-colored/clear in noninflammatory cases and cloudy/yellow in inflammatory ones.

SLE – LAB Dx

• Complete blood count (CBC) with differential• Serum creatinine• Urinalysis with microscopy• The CBC count may help to screen for

leukopenia, lymphopenia, anemia, and thrombocytopenia. Urinalysis and creatinine studies may be useful to screen for kidney disease.

SLE – LAB TEST

• The following are autoantibody tests used in the diagnosis of SLE[46] :

• ANA - Screening test; sensitivity 95%; not diagnostic without clinical features

• Anti-dsDNA - High specificity; sensitivity only 70%; level variable based on disease activity

• Anti-Sm - Most specific antibody for SLE; only 30-40% sensitivity

SCLERODERMA – LAB TEST

• Anti-RNA polymerase III, anti-topoisomerase I (also called anti-DNA topo 1) and anti-centromere antibodies (ACA) are also autoantibodies. Most patients with systemic scleroderma (but not localized scleroderma) have one or more of these autoantibodies.

Scleroderma- biopsy showing fibrosis

DERMATOMYOSITIS – Dx

• Magnetic resonance imaging (MRI). A scanner creates cross-sectional images of your muscles from data generated by a powerful magnetic field and radio waves.

• Electromyography. A doctor with specialized training inserts a thin needle electrode through the skin into the muscle to be tested. Electrical activity is measured as you relax or tighten the muscle, and changes in the pattern of electrical activity can confirm a muscle disease. The doctor can determine the distribution of the disease by testing different muscles.

• Muscle biopsy. A small piece of muscle tissue is removed surgically for laboratory analysis. In dermatomyositis, inflammatory cells surround and damage the capillary blood vessels in the muscle. A muscle biopsy may reveal inflammation in your muscles or other problems, like damage or infection. The tissue sample can also be examined for the presence of abnormal proteins and checked for enzyme deficiencies.

• Blood analysis. A blood test will let your doctor know if you have elevated levels of muscle enzymes, such as creatine kinase (CK) and aldolase. Increased CK and aldolase levels can indicate muscle damage. A blood test can also detect specific autoantibodies associated with different symptoms of dermatomyositis, which can help in determining the best medication and treatment.

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