A Case of Dermatomyositis

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A Case of Dermatomyositis

  1. 2. <ul><li>40 yr old mrs stella rani has come to the hospital with c/o difficulty in using all 4 limbs-last 7 months </li></ul><ul><li>Blackish discoloration face and hands-6 months </li></ul><ul><li>Difficulty in swallowing 2 months </li></ul><ul><li>Pain over the both great toes 1month </li></ul>
  2. 3. <ul><li>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 </li></ul><ul><li>h/o difficulty in getting up from squatting and lying posture, climbing the stairs -7months </li></ul><ul><li>Difficulty in lifting the hand above the head, combing the hair and bringing the food to mouth -6 months </li></ul><ul><li>h/o difficulty in lifting the head from the pillow-4 months </li></ul><ul><li>Difficulty in mixing the food- 2 months </li></ul><ul><li>Difficulty in holding the chapels last 2months </li></ul>
  3. 4. <ul><li>h/o hyper pigmentation over the face, chest and neck-6 months, non itchy </li></ul><ul><li>h/o dysphagia .- last 2 months , started with solid food now even for liquid, progressive, </li></ul><ul><li>mainly during initiation of swallowing and associated with throat pain </li></ul><ul><li>h/o pain over the both great toes and 2 nd toes for last 1month </li></ul><ul><li>h/o excessive hair loss + </li></ul>
  4. 5. <ul><li>No h/o difficulty in turning in bed from side to side </li></ul><ul><li>No h/o diurnal variation of weakness </li></ul><ul><li>No h/o twitching of muscles </li></ul><ul><li>No h/o sensory symptoms, cerebellar and posterior column symptoms </li></ul><ul><li>No h/o seizure </li></ul>
  5. 6. <ul><li>No h/o ptosis, diplopia </li></ul><ul><li>h/o dryness of mouth + </li></ul><ul><li>No h/o dryness of eyes </li></ul><ul><li>h/o cough with expectoration, white colour, scanty sputum </li></ul><ul><li>h/o dypnea on exertion + </li></ul><ul><li>No h/o fever </li></ul><ul><li>No h/o Photosensitivity </li></ul><ul><li>No h/o Altered bowel and bladder habits </li></ul><ul><li>h/o Head ache+ </li></ul><ul><li>No loss of appetite but loss of weight+ </li></ul>
  6. 7. <ul><li>PAST HISTORY </li></ul><ul><li>She had PT 10 years back, completed ATT </li></ul><ul><li>No H/O similar complaints in the past </li></ul><ul><li>No H/O DM,SHT,BA,CAD </li></ul><ul><li>FAMILY HISTORY </li></ul><ul><li>married, has 4 children, </li></ul><ul><li>no h/o spontaneous abortion </li></ul><ul><li>no other family member suffering from similar illness </li></ul><ul><li>Menstrual history </li></ul><ul><li>RMP 3/30 DAYS </li></ul><ul><li>TREATMENT HISTORY </li></ul><ul><li>not on any chronic treatment </li></ul><ul><li>no h/o drug allergy </li></ul>
  7. 8. <ul><li>Pt moderately built and nourished </li></ul><ul><li>Conscious ,oriented ,afebrile </li></ul><ul><li>pallor+ </li></ul><ul><li>No icterus/no cyanosis/no clubbing/no PE/no GLA </li></ul><ul><li>no thyromegaly </li></ul><ul><li>vitals -BP-150/90 mmHg, PR-86/min, RR-18/m regular, thoraco abdominal </li></ul>
  8. 9. <ul><li>Skin – dark red rusty erythematous lesions over the malar region, fore head, both shoulder, upper chest and back ( V sign, shawl sign present) </li></ul><ul><li>Erythematous lesion over the MCP and PIP- Gottron papules and sign + </li></ul><ul><li>Cold great toes with tenderness + </li></ul><ul><li>ORAL CAVITY- poor oral hygiene, ulcer over the left upper buccal mucosa , carie`s tooth </li></ul>
  9. 14. <ul><li>Higher function- normal </li></ul><ul><li>Cranial nerves- palatal movements equal on both side but sluggish, otherwise normal CN examination </li></ul><ul><li>Spino motor system </li></ul><ul><li>RT LT </li></ul><ul><li>bulk- generalized wasting+ </li></ul><ul><li>Arm 28cm 28cm </li></ul><ul><li>Fore arm 24cm 24cm </li></ul><ul><li>Thigh 42cm 42cm </li></ul><ul><li>Leg 24cm 24cm </li></ul>
  10. 15. <ul><li>Tone - normal on both side </li></ul><ul><li>Power - shoulder 2/5 both side </li></ul><ul><li>Elbow 3/5 both side </li></ul><ul><li>wrist 4/5 both side </li></ul><ul><li>hand grip good </li></ul><ul><li>hip 2/5 ,knee 3/5, ankle 4/5 </li></ul><ul><li>DTR preserved </li></ul><ul><li>Plantar b/l flexor , other superficial reflexes normal </li></ul><ul><li>Sensory system normal </li></ul><ul><li>No cerebellor sign </li></ul><ul><li>No involuntory movements </li></ul><ul><li>No fasiculation </li></ul><ul><li>Gait- waddling gait </li></ul>
  11. 16. <ul><li>CVS- </li></ul><ul><li>S1S2+, No murmur </li></ul><ul><li>RS- </li></ul><ul><li>NVBS+, No added sounds </li></ul><ul><li>ABDOMEN- </li></ul><ul><li>soft , Epigastric tenderness+ </li></ul><ul><li>No organomegaly no FF </li></ul>
  12. 17. <ul><li>Inflammatory myopathy </li></ul><ul><li>Dermatomyositis </li></ul><ul><li>or </li></ul><ul><li>Dermatomyositis with overlap syndrome </li></ul><ul><li>Anemia </li></ul><ul><li>SHT </li></ul><ul><li>Old PT </li></ul>
  13. 18. <ul><li>T.Prednisalone 40 mg od </li></ul><ul><li>T.Ranitidine 150mg bd </li></ul><ul><li>Inj.Ampicillin 1g iv tid </li></ul><ul><li>Inj.Deriphylline 2cc iv bd </li></ul><ul><li>T.Amlodipine 5mg od </li></ul><ul><li>Supportive measures </li></ul>
  14. 19. 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
  15. 21. <ul><li>Rheumatologist advised to transfer the patient to GGH for evaluation & management </li></ul>
  16. 26. <ul><li>CPK-35 RA factor-negative </li></ul><ul><li>ALT-316 CRP- negative </li></ul><ul><li>AST-114 ANA-POSITIVE </li></ul><ul><li>LDH-2461 anti dsDNA-NEGATIVE </li></ul><ul><li>CHL-262 </li></ul><ul><li>TGL-287 </li></ul>
  17. 27. <ul><li>ECG -WNL </li></ul><ul><li>ECHO -NORMAL STUDY </li></ul><ul><li>USG abdomen - normal study </li></ul><ul><li>X ray both hands-soft tissue swelling, no calcification, no osteoporotic lesion </li></ul><ul><li>X ray elbow-soft tissue swelling </li></ul><ul><li>HRCT chest- cystic ectasia of bronchi in the posterior segment of Rt upper lobe, </li></ul><ul><li>Ground glass opacity noted in the basal segments of both Lower Lobe L>R </li></ul>
  18. 28. <ul><li>DERMATOLOGY - confirm the DM </li></ul><ul><li>Neurology - inflammatory myopathy </li></ul><ul><li>EMG fixed on 13/4/10 </li></ul><ul><li>Cardiac evaluation- normal </li></ul><ul><li>Chest physician opinion- DM with ILD, old PT seqeule </li></ul><ul><li>MGE - UGI scopy normal, colonoscopy fixed on 19/3/10, barium swallow to be done </li></ul><ul><li>Dental opinion -chronic gingivitis, traumatic ulcer over the left buccal mucosa </li></ul><ul><li>ENT opinion - no growth in the pharynx, vocal cords normal </li></ul>
  19. 29. <ul><li>Inflammatory myopathy </li></ul><ul><li>Dermatomyositis </li></ul><ul><li>ILD </li></ul><ul><li>ANEMIA </li></ul><ul><li>SHT </li></ul><ul><li>OLD PT SEQEULE </li></ul>
  20. 30. <ul><li>Steroids </li></ul><ul><li>Methotrexate/cyclophosphamide </li></ul><ul><li>Hydroxy chloroquine </li></ul><ul><li>She is improving….. </li></ul>
  21. 31. Idiopathic InflammatoryMyopathies (IIM ) Heterogeneous group of autoimmune syndromes characterized by chronic muscle weakness and muscle inflammation, systemic complications
  22. 32. <ul><li>Adult polymyositis (PM) </li></ul><ul><li>Adult dermatomyositis (DM) </li></ul><ul><li>Juvenile myositis (DM >> PM) </li></ul><ul><li>Malignancy-associated myositis </li></ul><ul><li>Myositis in overlap with another rheumatic disease </li></ul><ul><li>Inclusion body myositis (IBM) </li></ul><ul><li>Other uncommon forms </li></ul>
  23. 33. <ul><li>Prevalence 1/100,000 </li></ul><ul><li>Estimated annual incidence of 1.9-7.7 per million persons </li></ul><ul><li>Women more commonly affected 2:1 </li></ul><ul><li>Peak age of onset 40-50 </li></ul>
  24. 34. <ul><li>The cause is unknown, but it may result from either a viral infection or an autoimmune reaction </li></ul><ul><li>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. </li></ul><ul><li>Genetic – HLA DR3,DR5,DR, a-TNF polymorphism </li></ul><ul><li>Immune – abnormal T cell activity </li></ul><ul><li>Infectious – viral agents, Toxoplasma, Borrelia </li></ul><ul><li>Drugs – Hydroxyurea, penicillamine, statin, quinidine, phenylbutazone </li></ul>
  25. 35. <ul><li>Dermatomyositis is probably caused by B cell and complement-mediated (terminal attack complex) vascular inflammation, </li></ul><ul><li>while polymyositis is caused by the direct cytotoxic effect of CD8 + lymphocytes on muscle. </li></ul><ul><li>However, other studies of cytokines suggest that some of the inflammatory processes may be similar. </li></ul><ul><li>A recent report has linked tumor necrosis factor (TNF) abnormalities with dermatomyositis. </li></ul>
  26. 36. <ul><li>Progressive, gradual (over many months), symmetric muscle weakness  difficulty with everyday tasks that require proximal muscles </li></ul><ul><li>Fine motor skills may be affected later in the disease course (but are common early in IBM) </li></ul><ul><li>Normal sensation </li></ul><ul><li>Pharyngeal and neck flexor muscles are often involved  may have dysphagia or head drop </li></ul><ul><li>May have respiratory involvement late in the presentation </li></ul><ul><li>Ocular and facial muscles are spared </li></ul><ul><li>Tendon reflexes preserved </li></ul><ul><li>Myalgia and muscle tenderness may occur, early </li></ul><ul><li>in the disease </li></ul>
  27. 38. <ul><ul><ul><li>Heliotrope rash : purple discoloration on the upper eyelids with peri orbital edema </li></ul></ul></ul><ul><ul><ul><li>Gottron’s papules : raised scaly violaceous rash over PIP and MCP joints </li></ul></ul></ul><ul><ul><ul><li>Erythematous rash : occurs on knees, elbows, neck and anterior chest ( V sign ), back and shoulders ( shawl sign ) </li></ul></ul></ul><ul><ul><ul><li>Raynaud’s phenomenon, subQ calcifications, periungal erythema </li></ul></ul></ul><ul><ul><ul><li>Mechanic`s hand - irregular, thickened, distorted lateral and palmar surface of fingers </li></ul></ul></ul>
  28. 45. <ul><ul><li>CD8 + T cells invade muscle fascicles(endo mysial invation), surround healthy muscle fibers and result in phagocytosis and necrosis </li></ul></ul>
  29. 46. <ul><ul><li>B cell and CD4+ T cell infiltrates within the perivascular or interfascicular space, antibodies activate C3 and MAC complex  activation of cytokines and chemokines </li></ul></ul>
  30. 47. <ul><li>Spinal muscular atrophy, ALS (use EMG to help) </li></ul><ul><li>Muscular dystrophies  develop over longer period </li></ul><ul><li>Guillain-Barre, polio, West Nile virus, neurotoxin  acute muscle weakness </li></ul><ul><li>AZT - causes a mitochondrial myopathy </li></ul><ul><li>Drug reaction- Amphotericin B, Heroin, Amiodarone, Colchicine, chronic laxative use, Glucocorticoids, Statins, Cyclosporine, Gemfibrozil, Alcohol, Cocaine </li></ul><ul><li>Steroid induced muscle weakness </li></ul><ul><li>Polymyalgia rheumatica , fibromyalgia  muscle tenderness, pain with movement </li></ul><ul><li>Connective tissue diseases </li></ul><ul><li>Endocrine –Hypo and hyperthyroidism </li></ul>
  31. 48. <ul><li>Muscle enzymes – CK, AST, ALT, LDH, aldolase </li></ul><ul><li>CK sometimes normal in active DM and IBM but always elevated in active PM </li></ul><ul><li>ESR and CRP are often normal and are not reliable indicator of disease activity </li></ul><ul><li>Antibodies </li></ul><ul><ul><li>positive ANA in > 75% </li></ul></ul><ul><ul><li>Anti Jo-1: found in 25%, 80% with this positive have lung involvement (antihistidyl transfer RNA [t-RNA] synthetase) </li></ul></ul><ul><ul><li>Anti Mi-2: found in 5-10%, more common in dermatomyositis </li></ul></ul><ul><ul><li>Anti –SRP associated with rapidly progressive PM </li></ul></ul><ul><li>EMG – </li></ul><ul><ul><li>short duration, low amplitude polyphasic units on voluntary activation </li></ul></ul><ul><ul><li>increased spontaneous activity with fibrillations, complex repetitive discharges, positive sharp waves </li></ul></ul><ul><li>Muscle biopsy </li></ul><ul><li>MRI can detect early and patchy involvement, can guide biopy </li></ul>
  32. 49. <ul><li>Symmetric proximal muscle weakness </li></ul><ul><li>Elevation of serum muscle enzymes: CK, aldolase, AST, ALT, LDH </li></ul><ul><li>Abnormal electromyogram (EMG) </li></ul><ul><li>Characteristic muscle pathology – </li></ul><ul><ul><li>degeneration (breakdown) and regeneration (healing) </li></ul></ul><ul><ul><li>inflammatory cells attacking muscle </li></ul></ul>
  33. 50. <ul><li>Muscle pain </li></ul><ul><li>Positive Anti-Jo-1 </li></ul><ul><li>Arthritis/arthralgia </li></ul><ul><li>Systemic inflammatory signs: fever, CRP, ESR </li></ul><ul><li>4+ of them = PM </li></ul><ul><li>Skin rash of DM: Gottron’s papules or sign, heliotrope rash </li></ul><ul><li>4+ and skin rash = DM </li></ul>
  34. 51. <ul><li>Extramuscular manifestations – </li></ul><ul><ul><li>Systemic : fever, weight loss, arthralgias and raynaud”s phenomenon </li></ul></ul><ul><ul><li>Joint contractures common in juvanile DM </li></ul></ul><ul><ul><li>GI : dysphagia (upper 1/3 striated muscle) </li></ul></ul><ul><ul><li>Cardiac : AV conduction defects, tachyarrhythmias, dilated cardiomyopathy, CHF </li></ul></ul><ul><ul><li>Pulmonary : respiratory muscle weakness, interstitial lung disease </li></ul></ul><ul><ul><li>calcinosis -Subcutanious calcifications common in JDM </li></ul></ul>
  35. 52. <ul><li>Assoctation with Malignancy </li></ul><ul><ul><li>Most common include ovarian, breast carcinoma, melanoma, colon, non-Hodgkin’s lymphoma </li></ul></ul><ul><ul><li>Nasopharyngeal cancer common in Asians </li></ul></ul><ul><ul><li>Search for an occult malignancy in all adult cases of DM should be done. </li></ul></ul>
  36. 53. <ul><li>Can associated with systemic sclerosis or mixed connective tissue disease </li></ul><ul><li>Very rarely with RA , SLE or sjogren`s </li></ul><ul><li>DM with SS will have a specific anti nuclear antibody, anti-pm/scl, directed against a nucleolar protein complex </li></ul>
  37. 54. <ul><li>Antisynthetase syndrome </li></ul><ul><li>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 ) </li></ul><ul><li>Amyopthic DM or Dermatomyositis sine myositis </li></ul><ul><li>Skin lesion without muscle involvement </li></ul><ul><li>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 . </li></ul>
  38. 55. <ul><li>Juvenile dermatomyositis is a systemic vasculopathy, affecting mainly the skin and muscle. </li></ul><ul><li>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. </li></ul><ul><li>Calcinosis is an additional feature that is present in juvenile dermatomyositis </li></ul><ul><li>Juvenile dermatomyositis is not associated with development of malignancies, unlike adult dermatomyositis. </li></ul>
  39. 56. <ul><li>Other features </li></ul><ul><li>Vasculitis and skin ulceration </li></ul><ul><ul><li>Myocarditis with arrhythmias can occur </li></ul></ul><ul><ul><li>Arthralgia/arthritis with contractures </li></ul></ul><ul><ul><li>Muscular contractures  decreased joint mobility </li></ul></ul><ul><ul><li>Gastrointestinal dysfunction </li></ul></ul><ul><ul><li>Pulmonary involvement </li></ul></ul><ul><ul><li>Calcinosis (after 1—2 years ) </li></ul></ul>
  40. 58. <ul><li>White, male, and over the age of 50 </li></ul><ul><li>Onset more insidious than PM or DM –over years </li></ul><ul><li>Asymmetric distal motor weakness is common </li></ul><ul><li>CK- mostly normal </li></ul><ul><li>Muscle biopsy-intracellular vacuoles with tubular or filamentous inclusions in the nucleus or cytoplasm </li></ul>
  41. 59. <ul><li>Glucocorticoids: start with high dose prednisone 1 mg/kg/day, taper after 4 weeks until lowest dose possible reached </li></ul><ul><ul><li>Caution as long term use may cause muscle weakness, osteoporosis </li></ul></ul><ul><ul><li>75% will require additional medications </li></ul></ul><ul><li>Immunosuppressants : Azathioprine-3mg/kg daily, Methotrexate-7.5mg/wk, Cyclophosphamide-0.5-1g/monthly, Chlorambucil, Cyclosporine, Mycophenolate mofetil-2.5mg/day </li></ul><ul><li>Immuno modulators – IV Ig –improved not only strength but also the underlying immuno pathology, benefit short lived, Repeated infusion q6-8 weeks </li></ul><ul><li>Dose 2g/kg divided over2-5 days per course </li></ul><ul><li>Plasmapheresis – no statistical difference between those given immunosuppressants and those who received plasmapheresis </li></ul><ul><li>For skin rashes - Limit sun exposure, hydroxychloroquine </li></ul>
  42. 60. <ul><li>For both DM and PM, 5 year survival is 95%, 10 year survival is 84% </li></ul><ul><li>Death is usually due to pulmonary, cardiac or systemic complications </li></ul><ul><li>Poor prognostic factors – older age at diagnosis, ethnicity, bulbar involvement, delayed treatment, cardiac and pulmonary involvement </li></ul><ul><li>No correlation with prognosis - CPK level, grade of disability and degree of muscle weakness at presentation </li></ul>
  43. 61. <ul><li>Choy and Isenberg. “ Treatment of dermatomyositis and polymyositis”. Rheumatology . 2002; 41: 7-13. </li></ul><ul><li>Dourmishev and Wollina. “ Dermatomyositis: immunopathologic study of skin lesions”. Acta Dermatoven APA. Vol 15, No 1. 2006. </li></ul><ul><li>Harrison’s Internal Medicine, 17 th edition . </li></ul><ul><li>Miller, M. “Clinical manifestations and diagnosis of adult dermatomyositis and polymyositis” UpToDate Online. Last updated February 15, 2008. </li></ul><ul><li>Miller, M. and Rudnicki, S. “Initial treatment of dermatomyositis and polymyositis in adults.” UpToDate Online. Last updated June 5, 2008. </li></ul><ul><li>www.neuropathologyweb.org . Northeastern Ohio Universities College of Medicine. </li></ul>

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