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A Case of Dermatomyositis

A Case of Dermatomyositis






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    A Case of Dermatomyositis A Case of Dermatomyositis Presentation Transcript

      • 40 yr old mrs stella rani has come to the hospital with c/o difficulty in using all 4 limbs-last 7 months
      • Blackish discoloration face and hands-6 months
      • Difficulty in swallowing 2 months
      • Pain over the both great toes 1month
      • She was apparently normal 7 months ago when developed pain over the both thighs followed by fever, she took treatment, fever subsided in 3 days but she noticed weakness of both legs, mainly in the proximal region , which is insidious in onset and slowly progressive in nature
      • h/o difficulty in getting up from squatting and lying posture, climbing the stairs -7months
      • Difficulty in lifting the hand above the head, combing the hair and bringing the food to mouth -6 months
      • h/o difficulty in lifting the head from the pillow-4 months
      • Difficulty in mixing the food- 2 months
      • Difficulty in holding the chapels last 2months
      • h/o hyper pigmentation over the face, chest and neck-6 months, non itchy
      • h/o dysphagia .- last 2 months , started with solid food now even for liquid, progressive,
      • mainly during initiation of swallowing and associated with throat pain
      • h/o pain over the both great toes and 2 nd toes for last 1month
      • h/o excessive hair loss +
      • No h/o difficulty in turning in bed from side to side
      • No h/o diurnal variation of weakness
      • No h/o twitching of muscles
      • No h/o sensory symptoms, cerebellar and posterior column symptoms
      • No h/o seizure
      • No h/o ptosis, diplopia
      • h/o dryness of mouth +
      • No h/o dryness of eyes
      • h/o cough with expectoration, white colour, scanty sputum
      • h/o dypnea on exertion +
      • No h/o fever
      • No h/o Photosensitivity
      • No h/o Altered bowel and bladder habits
      • h/o Head ache+
      • No loss of appetite but loss of weight+
      • She had PT 10 years back, completed ATT
      • No H/O similar complaints in the past
      • No H/O DM,SHT,BA,CAD
      • married, has 4 children,
      • no h/o spontaneous abortion
      • no other family member suffering from similar illness
      • Menstrual history
      • RMP 3/30 DAYS
      • not on any chronic treatment
      • no h/o drug allergy
      • Pt moderately built and nourished
      • Conscious ,oriented ,afebrile
      • pallor+
      • No icterus/no cyanosis/no clubbing/no PE/no GLA
      • no thyromegaly
      • vitals -BP-150/90 mmHg, PR-86/min, RR-18/m regular, thoraco abdominal
      • Skin – dark red rusty erythematous lesions over the malar region, fore head, both shoulder, upper chest and back ( V sign, shawl sign present)
      • Erythematous lesion over the MCP and PIP- Gottron papules and sign +
      • Cold great toes with tenderness +
      • ORAL CAVITY- poor oral hygiene, ulcer over the left upper buccal mucosa , carie`s tooth
      • Higher function- normal
      • Cranial nerves- palatal movements equal on both side but sluggish, otherwise normal CN examination
      • Spino motor system
      • RT LT
      • bulk- generalized wasting+
      • Arm 28cm 28cm
      • Fore arm 24cm 24cm
      • Thigh 42cm 42cm
      • Leg 24cm 24cm
      • Tone - normal on both side
      • Power - shoulder 2/5 both side
      • Elbow 3/5 both side
      • wrist 4/5 both side
      • hand grip good
      • hip 2/5 ,knee 3/5, ankle 4/5
      • DTR preserved
      • Plantar b/l flexor , other superficial reflexes normal
      • Sensory system normal
      • No cerebellor sign
      • No involuntory movements
      • No fasiculation
      • Gait- waddling gait
      • CVS-
      • S1S2+, No murmur
      • RS-
      • NVBS+, No added sounds
      • ABDOMEN-
      • soft , Epigastric tenderness+
      • No organomegaly no FF
      • Inflammatory myopathy
      • Dermatomyositis
      • or
      • Dermatomyositis with overlap syndrome
      • Anemia
      • SHT
      • Old PT
      • T.Prednisalone 40 mg od
      • T.Ranitidine 150mg bd
      • Inj.Ampicillin 1g iv tid
      • Inj.Deriphylline 2cc iv bd
      • T.Amlodipine 5mg od
      • Supportive measures
    • HB 9.4 RBS 122 TC 9200 UREA 18 DC P60L35E5 CREATININE 0.9 ESR 17/32 Na 136 MCV 86.7 K 3.9 MCH 25.5 Cl 98 MCHC 29.7 HCO3 23 PCV 24 PLATELETS 1.78LAcs URINE ROUTINE- ALB/sug-nil DEP-1-2 pc RBC 4.16 million
      • Rheumatologist advised to transfer the patient to GGH for evaluation & management
      • CPK-35 RA factor-negative
      • ALT-316 CRP- negative
      • AST-114 ANA-POSITIVE
      • LDH-2461 anti dsDNA-NEGATIVE
      • CHL-262
      • TGL-287
      • ECG -WNL
      • USG abdomen - normal study
      • X ray both hands-soft tissue swelling, no calcification, no osteoporotic lesion
      • X ray elbow-soft tissue swelling
      • HRCT chest- cystic ectasia of bronchi in the posterior segment of Rt upper lobe,
      • Ground glass opacity noted in the basal segments of both Lower Lobe L>R
      • DERMATOLOGY - confirm the DM
      • Neurology - inflammatory myopathy
      • EMG fixed on 13/4/10
      • Cardiac evaluation- normal
      • Chest physician opinion- DM with ILD, old PT seqeule
      • MGE - UGI scopy normal, colonoscopy fixed on 19/3/10, barium swallow to be done
      • Dental opinion -chronic gingivitis, traumatic ulcer over the left buccal mucosa
      • ENT opinion - no growth in the pharynx, vocal cords normal
      • Inflammatory myopathy
      • Dermatomyositis
      • ILD
      • ANEMIA
      • SHT
      • Steroids
      • Methotrexate/cyclophosphamide
      • Hydroxy chloroquine
      • She is improving…..
    • Idiopathic InflammatoryMyopathies (IIM ) Heterogeneous group of autoimmune syndromes characterized by chronic muscle weakness and muscle inflammation, systemic complications
      • Adult polymyositis (PM)
      • Adult dermatomyositis (DM)
      • Juvenile myositis (DM >> PM)
      • Malignancy-associated myositis
      • Myositis in overlap with another rheumatic disease
      • Inclusion body myositis (IBM)
      • Other uncommon forms
      • Prevalence 1/100,000
      • Estimated annual incidence of 1.9-7.7 per million persons
      • Women more commonly affected 2:1
      • Peak age of onset 40-50
      • The cause is unknown, but it may result from either a viral infection or an autoimmune reaction
      • Some cases of DM are a paraneoplastic phenomenon , indicating the presence of cancer and is usually pre-existent, with removal of the cancer resulting in remission of the DM.
      • Genetic – HLA DR3,DR5,DR, a-TNF polymorphism
      • Immune – abnormal T cell activity
      • Infectious – viral agents, Toxoplasma, Borrelia
      • Drugs – Hydroxyurea, penicillamine, statin, quinidine, phenylbutazone
      • Dermatomyositis is probably caused by B cell and complement-mediated (terminal attack complex) vascular inflammation,
      • while polymyositis is caused by the direct cytotoxic effect of CD8 + lymphocytes on muscle.
      • However, other studies of cytokines suggest that some of the inflammatory processes may be similar.
      • A recent report has linked tumor necrosis factor (TNF) abnormalities with dermatomyositis.
      • Progressive, gradual (over many months), symmetric muscle weakness  difficulty with everyday tasks that require proximal muscles
      • Fine motor skills may be affected later in the disease course (but are common early in IBM)
      • Normal sensation
      • Pharyngeal and neck flexor muscles are often involved  may have dysphagia or head drop
      • May have respiratory involvement late in the presentation
      • Ocular and facial muscles are spared
      • Tendon reflexes preserved
      • Myalgia and muscle tenderness may occur, early
      • in the disease
          • Heliotrope rash : purple discoloration on the upper eyelids with peri orbital edema
          • Gottron’s papules : raised scaly violaceous rash over PIP and MCP joints
          • Erythematous rash : occurs on knees, elbows, neck and anterior chest ( V sign ), back and shoulders ( shawl sign )
          • Raynaud’s phenomenon, subQ calcifications, periungal erythema
          • Mechanic`s hand - irregular, thickened, distorted lateral and palmar surface of fingers
        • CD8 + T cells invade muscle fascicles(endo mysial invation), surround healthy muscle fibers and result in phagocytosis and necrosis
        • B cell and CD4+ T cell infiltrates within the perivascular or interfascicular space, antibodies activate C3 and MAC complex  activation of cytokines and chemokines
      • Spinal muscular atrophy, ALS (use EMG to help)
      • Muscular dystrophies  develop over longer period
      • Guillain-Barre, polio, West Nile virus, neurotoxin  acute muscle weakness
      • AZT - causes a mitochondrial myopathy
      • Drug reaction- Amphotericin B, Heroin, Amiodarone, Colchicine, chronic laxative use, Glucocorticoids, Statins, Cyclosporine, Gemfibrozil, Alcohol, Cocaine
      • Steroid induced muscle weakness
      • Polymyalgia rheumatica , fibromyalgia  muscle tenderness, pain with movement
      • Connective tissue diseases
      • Endocrine –Hypo and hyperthyroidism
      • Muscle enzymes – CK, AST, ALT, LDH, aldolase
      • CK sometimes normal in active DM and IBM but always elevated in active PM
      • ESR and CRP are often normal and are not reliable indicator of disease activity
      • Antibodies
        • positive ANA in > 75%
        • Anti Jo-1: found in 25%, 80% with this positive have lung involvement (antihistidyl transfer RNA [t-RNA] synthetase)
        • Anti Mi-2: found in 5-10%, more common in dermatomyositis
        • Anti –SRP associated with rapidly progressive PM
      • EMG –
        • short duration, low amplitude polyphasic units on voluntary activation
        • increased spontaneous activity with fibrillations, complex repetitive discharges, positive sharp waves
      • Muscle biopsy
      • MRI can detect early and patchy involvement, can guide biopy
      • Symmetric proximal muscle weakness
      • Elevation of serum muscle enzymes: CK, aldolase, AST, ALT, LDH
      • Abnormal electromyogram (EMG)
      • Characteristic muscle pathology –
        • degeneration (breakdown) and regeneration (healing)
        • inflammatory cells attacking muscle
      • Muscle pain
      • Positive Anti-Jo-1
      • Arthritis/arthralgia
      • Systemic inflammatory signs: fever, CRP, ESR
      • 4+ of them = PM
      • Skin rash of DM: Gottron’s papules or sign, heliotrope rash
      • 4+ and skin rash = DM
      • Extramuscular manifestations –
        • Systemic : fever, weight loss, arthralgias and raynaud”s phenomenon
        • Joint contractures common in juvanile DM
        • GI : dysphagia (upper 1/3 striated muscle)
        • Cardiac : AV conduction defects, tachyarrhythmias, dilated cardiomyopathy, CHF
        • Pulmonary : respiratory muscle weakness, interstitial lung disease
        • calcinosis -Subcutanious calcifications common in JDM
      • Assoctation with Malignancy
        • Most common include ovarian, breast carcinoma, melanoma, colon, non-Hodgkin’s lymphoma
        • Nasopharyngeal cancer common in Asians
        • Search for an occult malignancy in all adult cases of DM should be done.
      • Can associated with systemic sclerosis or mixed connective tissue disease
      • Very rarely with RA , SLE or sjogren`s
      • DM with SS will have a specific anti nuclear antibody, anti-pm/scl, directed against a nucleolar protein complex
      • Antisynthetase syndrome
      • A subset of pts with PM and DM can have a group of findings including inflammatory arthritis, Raynaud`s phenomenon, ILD associated with certain auto antibodies( anti jo-1 )
      • Amyopthic DM or Dermatomyositis sine myositis
      • Skin lesion without muscle involvement
      • another subset of patients with dermatomyositis have controlled myopathy but continue to have severe and sometimes debilitating skin disease; this condition has been termed postmyopathic dermatomyositis .
      • Juvenile dermatomyositis is a systemic vasculopathy, affecting mainly the skin and muscle.
      • It differs from the adult form of dermatomyositis by the presence of vasculitis of the small blood vessels, which can involve the gastrointestinal tract and myocardium , besides skin and muscle.
      • Calcinosis is an additional feature that is present in juvenile dermatomyositis
      • Juvenile dermatomyositis is not associated with development of malignancies, unlike adult dermatomyositis.
      • Other features
      • Vasculitis and skin ulceration
        • Myocarditis with arrhythmias can occur
        • Arthralgia/arthritis with contractures
        • Muscular contractures  decreased joint mobility
        • Gastrointestinal dysfunction
        • Pulmonary involvement
        • Calcinosis (after 1—2 years )
      • White, male, and over the age of 50
      • Onset more insidious than PM or DM –over years
      • Asymmetric distal motor weakness is common
      • CK- mostly normal
      • Muscle biopsy-intracellular vacuoles with tubular or filamentous inclusions in the nucleus or cytoplasm
      • Glucocorticoids: start with high dose prednisone 1 mg/kg/day, taper after 4 weeks until lowest dose possible reached
        • Caution as long term use may cause muscle weakness, osteoporosis
        • 75% will require additional medications
      • Immunosuppressants : Azathioprine-3mg/kg daily, Methotrexate-7.5mg/wk, Cyclophosphamide-0.5-1g/monthly, Chlorambucil, Cyclosporine, Mycophenolate mofetil-2.5mg/day
      • Immuno modulators – IV Ig –improved not only strength but also the underlying immuno pathology, benefit short lived, Repeated infusion q6-8 weeks
      • Dose 2g/kg divided over2-5 days per course
      • Plasmapheresis – no statistical difference between those given immunosuppressants and those who received plasmapheresis
      • For skin rashes - Limit sun exposure, hydroxychloroquine
      • For both DM and PM, 5 year survival is 95%, 10 year survival is 84%
      • Death is usually due to pulmonary, cardiac or systemic complications
      • Poor prognostic factors – older age at diagnosis, ethnicity, bulbar involvement, delayed treatment, cardiac and pulmonary involvement
      • No correlation with prognosis - CPK level, grade of disability and degree of muscle weakness at presentation
      • Choy and Isenberg. “ Treatment of dermatomyositis and polymyositis”. Rheumatology . 2002; 41: 7-13.
      • Dourmishev and Wollina. “ Dermatomyositis: immunopathologic study of skin lesions”. Acta Dermatoven APA. Vol 15, No 1. 2006.
      • Harrison’s Internal Medicine, 17 th edition .
      • Miller, M. “Clinical manifestations and diagnosis of adult dermatomyositis and polymyositis” UpToDate Online. Last updated February 15, 2008.
      • Miller, M. and Rudnicki, S. “Initial treatment of dermatomyositis and polymyositis in adults.” UpToDate Online. Last updated June 5, 2008.
      • www.neuropathologyweb.org . Northeastern Ohio Universities College of Medicine.