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Dermatomyositis PPT
- 1. DERMATOMYOSITIS TITILOPE ADEEYO, GROUP25, 5th YEAR 9thSEMESTER, HOSPITAL THERAPY
- 2. • The inflammatorymyopathies represent the largest group of acquired and potentially treatable causes of skeletal muscle weakness. They are classified into three major groups: • Polymyositis (PM), • Dermatomyositis (DM), and • Inclusion body myositis (IBM).
- 3. DEFINITION • Dermatomyositis isa rare disease that causes muscle weakness and rashes on your skin. It’s a form of myopathy. It can also cause severe symptoms that affect your ability to breathe and swallow. • DM is a form of polymyositis that affects your skin in addition to your muscles. • DM affects both children and adults and women more often than men.
- 5. CLASSIFICATION 1. Adult dermatomyositis: •Classic DM • Classic DM with malignancy • Classic DM with associated connective tissue disease • Clinically amyopathic DM(CADM) 2. Juvenile DM(JDM) • Classic JDM • Clinically amyopathic JDM
- 7. ETIOLOGY • Cause isnot Known • For some reason, the body's immune system turns against its own muscles and damages muscle tissue in an autoimmune process. In dermatomyositis, these cells attack the small blood vessels that supply muscles and skin. • Genetic factors: Associated with HLADR3,DR5, DR7 • Viral infections: Viruses such as Coxsackie B, enteroviruses, and parvoviruses may be potential triggers for autoimmunity. • Environmental factors: high intensity ultraviolet (UV) radiation, pollution .
- 8. CLINICAL PRESENTATION 1. SKIN •The rash may consist of a blue-purple discoloration on the upper eyelids with edema (heliotrope rash), a flat red rash on the face and upper trunk, and erythema of the knuckles with a raised violaceous scaly eruption (Gottron's sign). • The erythematous rash can also occur on other body surfaces, including the knees, elbows, malleoli, neck and anterior chest (often in a V sign), or back and shoulders (shawl sign), and may worsen after sun exposure. • The cuticles may be irregular, thickened, and distorted,and the lateral and palmar areas of the fingers may become rough and cracked, with irregular, "dirty" horizontal lines, resembling mechanic's hands.
- 9. Gottron's papules onfinger joints Gottron's bumps on a person with juvenile DM
- 10. Heliotrope with swellingaround the eyes Severe rash on the hands, extending up the forearm
- 11. Shawl sign Vsign
- 12. 2. MUSCLE • DMpresent as progressive and symmetric muscle weakness. • Patients usually report increasing difficulty with everyday tasks requiring the use of proximal muscles, such as getting up from a chair, climbing steps, stepping onto a curb, lifting objects, or combing hair. • Distal muscles, such as buttoning a shirt, sewing, knitting, or writing, are affected only late in the course of DM. EXTRAMUSCULAR MANIFESTATIONS: • Systemic symptoms like fever, malaise, weakness • Joint contractures, Arthralgias, synovitis, or deforming arthropathy with subluxation in the interphalangeal joints • Subcutaneous calcifications • Cardiac disturbances • Pulmonary dysfunction
- 13. LABORATORY DIAGNOSIS 1. Commonblood test: • Changes are not specific: increase in ESR arises seldom (mainly at development of systemic manifestations).Biochemical assay:-CPK – the most sensitive and specific marker of muscular inflammation. The activity of CPK to some extent correlates with severity of muscle weakness. Increase in MB fraction CPK reflects damage of muscles, but not a myocardium. • The important diagnostic value has identification of aldolase, AST, ALT, and lactate dehydrogenase. • The activity of enzymes should be defined before carrying out a needle electromyography (EMG) (nonspecific increase in concentration of enzymes). 2. Immunological assay: • Antinuclear antibodies (ANA) are found in 24-60% of DM patients, 16-40% – PM and at 20% of patients with a myositis with inclusions. In the presence of very high titers of ANA, the cross syndrome with other systemic diseases of connective tissue is available. • An important diagnostic value is the identification of myositis-specific (anti-Jo-1, anti-Mi-2, signal representing particles (SRP) etc.) and myositis-associated (anti- RNP, anti-Scl70, anti-centromere, antibodies against Smith antigen, anti-Ro (SSA) and anti-La (SSB) antibodies 3. Urine analysis: • A proteinuria is rare. Urine discoloration (red, brown) can be connected with myoglobin which is result of massive rhabdomyolysis(evidence level B).
- 14. Instrumental investigations Needle electrodeelectromyography. A triad of signs is typical for primary muscular inflammatory lesion: 1 - decrease of motor unit action average potentials and their amplitudes; 2 - polyphasy and pseudo-polyphasy; 3 - spontaneous activity of muscle fibers in relaxed condition. The normal electric activity in the patients who have never received Glucocorticoids allows excluding the diagnosis except those with amyopathic forms of disease. The needle electromyography is also used for assessment of treatment efficiency.212 MRI of muscles is a sensitive method of assessment of intensity of muscular inflammation. Ultrasonography. Ultrasonic muscular visualization is an additional noninvasive method of investigation of neuromuscular pathology. High resolution computer tomography (HRCT): identification of basal pneumosclerosis and interstitial pulmonary fibrosis, lung cancer exclusion (recommended to be performed by any patient of 40 years old or elder). Investigation of the external respiration function. The myositis- associated ILD is characterized by decrease of general capacity of lungs, functional residual capacity, forced vital capacity, forced expiratory volume for the first second, diffusion capacity of carbon monoxide. IIM diagnosing is based mainly on clinical findings, data obtained from past medical history, results of muscle biopsy and results of laboratory assays and electromyographic investigation.The inflammatory myopathy can be suspected in patients with symmetric proximal muscle weakness combined with skin rash or without it.
- 16. DIAGNOSTIC CRITERIA The diagnosisof dermatomyositis is based on five criteria, which are also used to differentially diagnose with respect to polymyositis: 1. Muscle weakness in both thighs or both upper arms 2. Using a blood test, finding higher levels of enzymes found in skeletal muscle , including creatinine kinase, aldolase, and glutamate oxaloaxecetate, pyruvate transaminases and lactate dehydrogenase 3. Using electromyography (testing of electric signalling in muscles), finding all three of: erratic, repetitive, high-frequency signals; short, low-energy signals between skeletal muscles and motor neurons that have multiple phases; and sharp activity when a needle is inserted into the muscle 4. Examining a muscle biopsy under a microscope and finding mononuclear white blood cell between the muscle cells, and finding abnormal muscle cell degeneration and regeneration, dying muscle cells, and muscle cells being consumed by other cells (phagocytosis ) 5. Rashes typical of dermatomyositis, which include heliotrope rash, Gottron sign, and Gottron papules. The fifth criterion is what differentiates dermatomyositis from polymyositis; the diagnosis is considered definite for dermatomyositis if three of items 1 through 4 are present in addition to 5, probable with any two in addition to 5, and possible if just one is present in addition to 5.
- 18. DIFFERENTIAL DIAGNOSIS • Subacuteor Chronic Progressive Muscle Weakness • Myofasciitis • Acute Muscle Weakness • Necrotizing Autoimmune Myositis • Drug-Induced Myopathies d-Penicillamine, procainamide, and statins. • Hyperacute Necrotizing Fasciitis/Myositis (Flesh-Eating Disease).
- 19. TREATMENT The main treatmentis the use of corticosteroid medicines. The dose of medicine is slowly tapered off as muscle strength improves. This takes about 4 to 6 weeks. You may stay on a low dose of a corticosteroid medicine after that. Medicines to suppress the immune system may be used to replace the corticosteroids. These drugs may include azathioprine, methotrexate or mycophenolate. Treatments that may be tried when disease that remains active in spite of these medicines are: • Intravenous gamma globulin • Biologic drugs When your muscles get stronger, your provider may tell you to slowly cut back on your doses. Many people with this condition must take a medicine called prednisone for the rest of their lives. If a cancer is causing the condition, the muscle weakness and rash may get better when the tumor is removed
- 20. OUTLOOK (PROGNOSIS) It isimportant to be followed by a health care provider when you have dermatomyositis. Severe illness can lead to disability and can sometimes result in death. Symptoms may go away completely in some people, such as children. The condition may be fatal in adults due to: • Severe muscle weakness • Malnutrition • Pneumonia • Lung failure The major causes of death with this condition are cancer and lung disease. People with lung disease with the anti-MDA-5 antibody have a poor prognosis in spite of current treatment.