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a case presentation of polymyositis

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intersting case of polymyositis

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a case presentation of polymyositis

  1. 1. CASE PRESENTATION BY DR. ISTIKHAR ALI SAJJAD PGR MUIII AHF
  2. 2. HISTORY My patient ABC W/O XYZ 30 years old female resident of Gojra, house wife by occupation admitted via MEW on 27/11/2013 with following complaints  Muscle pains-------- 2 months  Gen. body weakness----- 1 month  Difficulty in swallowing ----- 10 days
  3. 3. Muscle aches  Usual state of health 2 months back  Gradual in onset  From lower limbs, then upper limbs then whole body  Aggravated on trying to move limb and pressing the muscles  Progressive  There is also history of associated B/L knee joint pain but no inflammation associated with it
  4. 4. Weakness  Gradual in onset  Unable to rise up from sitting position, climbing stairs  Then unable to move lower limbs  and also difficulty in combing hair then even moving her upper limbs  Didn’t c/o any difficulty in breathing or neck movements  There is no history of any morning stiffness.
  5. 5. Difficulty in swallowing  For last 10 days difficulty in swallowing  Difficulty is more for solids than liquids  No h/o regurgitation, vomiting.
  6. 6. History  There is no history of any associated fever, respiratory tract or GIT infection in past few weeks, palpitation, heat or cold intolerance, any change in urine color, cough, sputum.  There is also no history of use of any drugs for a long period, also no history of any homeo or herbal medicine.  There is no h/o mouth ulcers alopacia, sun burns, change in color of finger tips in cold.
  7. 7. Past History  There is no significant past medical or surgical history.
  8. 8. Family History  There is no family history of such illness and diabetes.
  9. 9. Drug History  No known allergy  Not using any kind of medicine for longer period of time.
  10. 10. Socio economic history  Patient is a house wife and belongs to a low socio economic family
  11. 11. Personal history  Married for last 08 years  Never conceived  Not properly evaluated for infertility  Non smoker and non drinker
  12. 12. Menstrual History  She c/o oligo-menorrhea for last one year.  No h/o dysmenorrhea  No h/o dysprunea.
  13. 13. Examination  A middle aged ill looking female lying comfortably in the bed with I/V cannula at right arm, well cooperative during examination with following vitals – B.P 110/70 mmHg – Pulse 82/min – R.R 20/min – Temp. 98`F
  14. 14. General Physical Examination  No significant finding on GPE  No erythematous rash at face  Shawl sign -ve  Gottron sign -ve  Heliotrope rash –ve  No mechanic’s hands  No signs of cushing’s disease  No skin changes  No signs of hyper/hypothyroidism
  15. 15. Musculoskeletal & Nervous system  Well oriented in time place and person  GCS 15/15  Mild tenderness in muscles of lower thigh  Plantars B/L down going  Power 1/5 at proximal muscles, 3/5 at distal muscles of lower limbs  2/5 in proximal muscles of upper limb and 4/5 in lower limbs
  16. 16. Musculoskeletal & Nervous system  Bulk of muscles bilateral equal and normal  Tone was normal in all limbs  Reflexes are normal  All sensations intact  All cranial nerves intact (no neurological dysphagia)
  17. 17. Other Systemic Examination  Respiratory system: – Normal in shape, bilateral chest movements equal, and bilaterla air entery equal. On auscultation normal vesicular breathing with few bibasal inspiratory crackles not changing character with cough.  CVS – Apex beat in 5th intercostal space just lateral to mid clavicular line non taping, non heaving with S1+S2+0
  18. 18. Abdomen  Scaphoid, with umbilicus normal in shape and position, flanks not filled and no visible veins or stria  soft, non tender, no visceromegaly  No shifting dullness  Bowel sounds 3/min
  19. 19. Differential diagnosis  Polymyositis  Mixed connective tissue disease  Polymyalgia Rheumatica  Fibromyalgia  Subclinical Hyperthyroidism/hypothyroidism  Steroid induced myopathy  Gillian Berre syndrome  Hyper/hypo/normo kalemic periodic paralysis
  20. 20. INVESTIGATIONS  CBC – Hb 10.7 g/dl – WBC 10800 – PLT count 191000 – Neut. 88% – Poly. 10% – Eosin.1% – ESR 100 mm/1st hr
  21. 21.  Serum electrolytes – Na 139 – K 4.4 – Cl 102  RFTS – Urea 60 – Creatinine 1.2  LFTs – ALT 370 IU – AST 490 IU – S. bilirubin 0.7 (0.4D) – Alk. PO4 204
  22. 22.  Urine C/E – Albumin nil – Pus cells rare – RBC 9-10 – Crystals uric acid+ – Blood ++ – pH 7.0 – sp. Gravity 1010 – Casts nil
  23. 23. CPK 7047 LDH 2785
  24. 24.  ANA +ve  Anti dsDNA -ve  Anti jo-I Abs –ve  Anti RNP antibodies not available  HBsAg –ve  Anti HCV -ve
  25. 25.  CXR (PA view) was normal  USG abd and pelvis showed no abnormality  ECG normal
  26. 26.  NCS Normal  EMG showed – Increased insertional activity with fibrillation and +ve sharp waves especially in proximal muscles. There is early recruitment of motor units and interference pattern is early and full especially in lower limbs and proximal muscles. Motor unit size and duration is decreased – Impression: this study is in favour of inflammatory myopathy(polymyositis)
  27. 27.  Biopsy of Skeletal muscle sent  CA 125 sent
  28. 28. Final diagnosis Polymyositis
  29. 29. Treatment given  High protein diet  Physiotherapy  Tab prednisolon 55mg/day  Cap. Omeprazole 40mg/day  Tab. Paracetamol TDS  Bisphosphonates  Ca supplements
  30. 30. After 5 days of treatment  Power in proximal muscles of upper limb 4/5 (prev. 2/5)  Power in proximal muscles of lower limb 2/5 (prev. 1/5)  Before R serum CPK 7047  After R Serum CPK 5400
  31. 31.  Mammography advised  HRCT Chest advised  Referred to Gynaecology for proper work up of Infertility
  32. 32. POLYMYOSITIS  Polymyositis is an idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness; elevated skeletal muscle enzyme levels; and characteristic electromyography (EMG) and muscle biopsy findings
  33. 33. TYPES Bohan and Peter classify the idiopathic inflammatory myopathies as follows : I - Primary idiopathic polymyositis II - Primary idiopathic dermatomyositis III - Polymyositis or dermatomyositis associated with malignancy[3] IV - Childhood polymyositis or dermatomyositis V - Polymyositis or dermatomyositis associated with another connective-tissue disease VI - Inclusion body myositis VII - Miscellaneous (eg, eosinophilic myositis, myositis ossificans, focal myositis, giant cell myositis)
  34. 34. MANIFESTATIONS  Constitutional manifestations  Muscular manifestations  Pulmonary manifestations  Cardiac manifestations  Joint involvement  Gastrointestinal manifestations  Renal manifestations  Cutaneous manifestations  Inclusion body myositis  Malignancies
  35. 35. INVESTIGATIONS  CBC  CPK  LDH  SGPT  SGOT  Urine C/E  Antio myosite specific antibodies  ANA  RA  ENA profile  EMG  Muscle Biopsy  CXR  ECG  Mammography
  36. 36. Treatment  Prednisone – first-line treatment of choice – 1 mg/kg/day, either as a single or divided dose. – This high dose is usually continued for 4-8 weeks, until the CK level returns to reference ranges. – Taper prednisone by 5-10 mg on a monthly basis until the lowest dose that controls the disease is reached.
  37. 37. Immunosuppressants  Indicated in patients who do not improve with steroids within a reasonable period (ie, 4 wk) or in whom adverse effects from corticosteroids develop.  Patients with poor prognostic indicators, such as dysphagia or dysphonia, are likely to require immunosuppressive agents.  Methotrexate is the second-line agent.  Patients with inclusion body myositis usually respond poorly to corticosteroids and immunosuppressive agents.
  38. 38.  Intravenous immunoglobulin (IVIG)  Tumor necrosis factor (TNF) inhibitors  Rituximab  Interferon alfa-2a
  39. 39.  Diet – Patients with polymyositis may benefit from a high-protein diet. Monitor patients to avoid excessive weight gain due to corticosteroid use.  Activity – Encourage patients with polymyositis to start a supervised exercise program early in the disease course. – During the acute stage of polymyositis, patients may benefit from heat therapy, passive range-of- motion exercises, and splints to avoid contractures.

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