Poly myositis and dermatomyositis Are acquired chronic inflammatory

Polymyositis (PM) and dermatomyositis (DM) -adult polymyositis -adult dermatomyositis -adult polymyositis, dermatomyositis with malignancies

-Mascular weakness is the dominant feature of myositis syndromes -Symmetryical -Proximal muscle of extremities,

-Lower extremities characterized by. An inability to climb stairs. To arise from low seat.

Adult polymysitis (PM) - Women: men 3: 1 The onset can be insidious, over

-Malaise- weight loss and fever can develop during the acute phase -The major feature

-Involvement of pharyngeal, laryngeal and respiratory muscles can lead to dysphagia , dysphonia and

Adult dermatomyositis -more in women -heliotrope rash and perorbital oedema -gottron rash -ulcerative vascuilitis

Dermatomyositis: heliotrope rash Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Dermatomyositis: diffuse facial erythema Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Dermatomyositis: rash, chest Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Dermatomyositis: edema and rash, hand Copyright © 1972 -2004 American College of Rheumatology Slide

Dermatomyositis: erythematous lesions, hands Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Dermatomyosistis: rash, hands Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Dermatomyositis: “mechanic’s hands” Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Dermatomyosistis: periungual involvement Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Dermatomyositis: rash, knees Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Dermatomyositis: subcutaneous calcification, knees Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Dermatomyositis: calcinosis, thigh (radiograph) Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Associated with malignancies -The associated cancer may not become apparent for 2 -3 years.

Childhood dermatomyositis -Age : 4 -10 years -Subcutaneous calcification maybe widespread and severe -Recurrent

Inclusion Body Myositis General Features Most common acquired muscle disease over the age of

$Clinical Features of IBM • Consider IBM when confronted with refractory polymyositis patient •$

Inclusion body myositis Males affected more than females Age of onset usually greater than

Polymyositis: differential diagnosis Polymyositis and dermatomyositis Hypothyroidism Drug-induced myopathies Corticosteroids, colchicine, HMG-Co. A reductase

Polymyositis: differential diagnosis, cont’d Metabolic myopathies Disorders of carbohydrate and lipid metabolism Electrolyte disturbances

Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Neurological disease -Asymmetrical weakness -distal extremety inovlvement -altered sesorium or abnormal cranialnerve function -myopathies

-Limb-girdle dystrophy positive family history upper and lower extremity proximal muscle weakness may begin

-Becker`s muscular dystrophy Similar but milder Patient able to walk beyond the age 16

Don’t Forget Patients with hypothyroidism can present with diffuse and nonspecific arthralgias and myalgias.

Think About Toxic Drug Reactions That Can Cause Musculoskeletal Pain Hydroxymethylglutaryl coenzyme A (HMG-Co.

Soft-Tissue Pain Syndromes: Fibromyalgia Widespread musculoskeletal pain Decreased pain threshold and tolerance May have

Common Causes of Proximal Muscle Weakness With Elevated CK Inflammatory myositis Noninflammatory myopathies Hypothyroidism

Myositis-specific antibodies ANTIBODY DISEASE ASSOCIATION PREVALENCE Anti-t. RNA synthetases Dermatomyositis, (Jo-1) interstitial lung disease,

-EMG -Needle muscle biopsy -MRI -screening for malignancy Copyright © 1972 -2004 American College

Proposed diagnostic criteria for polymyositis and dermatomyositis PM diagnosed as definite with 4 out

Polymyositis/Dermatomyositis -Therapy Prednisone 1– 2 mg/kg, as initial therapy Methotrexate or azathioprine is often

Treatment Options in Myositis Corticosteroids (prednisone) Immunosuppressive agents Methotrexate cyclosporine Azathioprine cyclophosphamide leflunomide chlorambucil

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Poly myositis and dermatomyositis Are acquired. chronic , inflammatory muscle condition of unknown cause -Age : most cases are in 5 th and 6 th decades of life. -Incidence: 0. 5 per 100, 000 population Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Polymyositis (PM) and dermatomyositis (DM) -adult polymyositis -adult dermatomyositis -adult polymyositis, dermatomyositis with malignancies -PM/DM in association with other connective tissue diseases -childhood DM. -inclusion body myositis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

-Mascular weakness is the dominant feature of myositis syndromes -Symmetryical -Proximal muscle of extremities, trunk and neck Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

-Lower extremities characterized by. An inability to climb stairs. To arise from low seat. Walking may be limited and gait may become poorly coordinated and waddling -Upper extremities. Limits lifting. Handing up clothes and combing the hair -Weakness of the anterior neck flexor muscles interferes with lifting the head from the supine position and arising from bed become difficult Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

-Malaise- weight loss and fever can develop during the acute phase -The major feature of PM is proximal muscle weakness which is progressive -There is wasting of the shoulder and pelvic girdle muscles with weakness -Pain and tenderness are uncommon -Patients have difficulty squatting going upstairs, raising from a chair raising their hands above the head and holding their head up. Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

-Involvement of pharyngeal, laryngeal and respiratory muscles can lead to dysphagia , dysphonia and respiratory failure. -Interstitial lung fibrosis -Arthrlgia 50% -Raynaud’s phenomenon -cardiac abnormalities (heart block , myocarditis, congestive heart failure) Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Adult dermatomyositis -more in women -heliotrope rash and perorbital oedema -gottron rash -ulcerative vascuilitis -subcutaneous calcinosis 25% Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Associated with malignancies -The associated cancer may not become apparent for 2 -3 years. -Recurrent or refractory dermatomyositis should prompt a search for occult malignancy -Lung, ovary, breast, stomach cancer can predate the onset of myositis particularly in males with dematomyositis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Childhood dermatomyositis -Age : 4 -10 years -Subcutaneous calcification maybe widespread and severe -Recurrent abdominal pain due to vascuilitis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Inclusion Body Myositis General Features Most common acquired muscle disease over the age of 50 Prevalence of 5 -10/million Affects men > women at 2 -3: 1 Average time from symptom onset to diagnosis is ~ 6 years Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

$Clinical Features of IBM • Consider IBM when confronted with refractory polymyositis patient •$

Clinical Features of IBM • Consider IBM when confronted with refractory polymyositis patient • Insidious onset of painless muscle weakness with slow progression • Tendency to distal (away from the trunk muscles) and asymmetric muscle involvement (“foot drop”) • Difficulty swallowing • Characteristic pattern of muscle atrophy (forearm flexors, muscles of hands, thigh) Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Inclusion body myositis Males affected more than females Age of onset usually greater than 50 Slowly progressive Distal and asymmetric muscle weakness Myopathic and neuropathic changes on EMG Mononuclear cell infiltrates and vacuoles containing amyloid on muscle biopsy Responds poorly to corticosteroids Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Polymyositis: differential diagnosis Polymyositis and dermatomyositis Hypothyroidism Drug-induced myopathies Corticosteroids, colchicine, HMG-Co. A reductase inhibitors, zidovudine, hydroxychloroquine, alcohol Infections Viral, toxoplasmosis, trichinosis, bacterial pyomyositis Connective tissue disorders Lupus, scleroderma, MCTD Systemic vasculitis PAN, Wegener’s granulomatosis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Polymyositis: differential diagnosis, cont’d Metabolic myopathies Disorders of carbohydrate and lipid metabolism Electrolyte disturbances Hypernatremia, hypokalemia, hypophosphatemia, hypocalcemia Inclusion body myositis Sarcoid myopathy Amyloid myopathy Neurologic disorders Myasthenia gravis, motor neuron disease, muscular dystrophy Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Neurological disease -Asymmetrical weakness -distal extremety inovlvement -altered sesorium or abnormal cranialnerve function -myopathies cause proximal and symmetrical weakness except (IBM, Mitochondrial myopathies, anti. SRP antibody) Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

-Limb-girdle dystrophy positive family history upper and lower extremity proximal muscle weakness may begin in the first through third decades of life. muscle wasting -Duchenn`es musclar dystrophy The age of 5 years Muscle weakness and wasting Hyperlordotic gait Psuedo hypertrophy of the calf muscle Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

-Becker`s muscular dystrophy Similar but milder Patient able to walk beyond the age 16 -Facioscapulo humeral Autosomal dominant Weakness begin in the facial muscle Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Don’t Forget Patients with hypothyroidism can present with diffuse and nonspecific arthralgias and myalgias. CKs may be elevated Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Think About Toxic Drug Reactions That Can Cause Musculoskeletal Pain Hydroxymethylglutaryl coenzyme A (HMG-Co. A) reductase inhibitor Zidovudine (AZT) Ethanol Clofibrate Cyclosporin A Penicillamine Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Soft-Tissue Pain Syndromes: Fibromyalgia Widespread musculoskeletal pain Decreased pain threshold and tolerance May have tenderness in specific regions (tender points) Associated fatigue, sleep, somatic complaints No objective inflammation seen on physical examination Normal laboratory findings Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Common Causes of Proximal Muscle Weakness With Elevated CK Inflammatory myositis Noninflammatory myopathies Hypothyroidism Hypokalemia Alcoholism Drugs AZT HMG-Co. A reductase inhibitors (the “statins”) Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Myositis-specific antibodies ANTIBODY DISEASE ASSOCIATION PREVALENCE Anti-t. RNA synthetases Dermatomyositis, (Jo-1) interstitial lung disease, “mechanic’s hands” 20% Anti-SRP (signal recognition protein) African-American women, poor prognosis Rare Anti-Mi-2 Older women, “shawl sign, ” 5% good prognosis PM/SCL Polymyositis/scleroderma overlap Rare Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Proposed diagnostic criteria for polymyositis and dermatomyositis PM diagnosed as definite with 4 out of 5 of the below criteria or probable with 3 out of 5 DM diagnosed as definite with rash plus 3 out of 4 of the below criteria or probable with rash plus 2 out of 4 criteria Symmetric proximal muscle weakness Elevated muscle enzymes (CPK, aldolase, transaminases, LDH) Myopathic EMG abnormalities Typical changes on muscle biopsy Typical rash of dermatomyositis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Polymyositis/Dermatomyositis -Therapy Prednisone 1– 2 mg/kg, as initial therapy Methotrexate or azathioprine is often added Intravenous immunoglobulin in rapidly progressive or refractory cases Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Treatment Options in Myositis Corticosteroids (prednisone) Immunosuppressive agents Methotrexate cyclosporine Azathioprine cyclophosphamide leflunomide chlorambucil mycophenolate mofetil IVIg Anti-TNF agents (etanercept, infliximab) “Biologic” response modifiers rituximab Oxandrolone (IBM) Other (stem cell transplant) Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.