Download - a case presentation of polymyositis
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CASE PRESENTATION
BYDR. ISTIKHAR ALI SAJJAD
PGR MUIII AHF
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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
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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
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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.
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Difficulty in swallowing For last 10 days difficulty in swallowing Difficulty is more for solids than liquids No h/o regurgitation, vomiting.
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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.
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Past History There is no significant past medical or
surgical history.
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Family History There is no family history of such illness
and diabetes.
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Drug History No known allergy Not using any kind of medicine for longer
period of time.
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Socio economic history Patient is a house wife and belongs to a low
socio economic family
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Personal history Married for last 08 years Never conceived Not properly evaluated for infertility Non smoker and non drinker
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Menstrual History She c/o oligo-menorrhea for last one year. No h/o dysmenorrhea No h/o dysprunea.
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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
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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
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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
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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)
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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
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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
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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
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INVESTIGATIONS CBC
– Hb 10.7 g/dl– WBC 10800– PLT count 191000– Neut. 88%– Poly. 10%– Eosin.1%– ESR 100 mm/1st hr
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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
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Urine C/E– Albumin nil– Pus cells rare– RBC 9-10– Crystals uric acid+– Blood ++– pH 7.0– sp. Gravity 1010– Casts nil
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CPK 7047
LDH 2785
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ANA +ve Anti dsDNA -ve Anti jo-I Abs –ve Anti RNP antibodies not available HBsAg –ve Anti HCV -ve
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CXR (PA view) was normal USG abd and pelvis showed no abnormality ECG normal
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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)
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Biopsy of Skeletal muscle sent CA 125 sent
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Final diagnosis
Polymyositis
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Treatment given High protein diet Physiotherapy Tab prednisolon 55mg/day Cap. Omeprazole 40mg/day Tab. Paracetamol TDS Bisphosphonates Ca supplements
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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
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Mammography advised HRCT Chest advised Referred to Gynaecology for proper work
up of Infertility
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LITERATURE REVIEW
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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
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TYPES Bohan and Peter classify the idiopathic inflammatory myopathies as follows :I - Primary idiopathic polymyositisII - Primary idiopathic dermatomyositisIII - Polymyositis or dermatomyositis associated with malignancy[3]
IV - Childhood polymyositis or dermatomyositisV - Polymyositis or dermatomyositis associated with another connective-tissue diseaseVI - Inclusion body myositisVII - Miscellaneous (eg, eosinophilic myositis, myositis ossificans, focal myositis, giant cell myositis)
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MANIFESTATIONS Constitutional manifestations Muscular manifestations Pulmonary manifestations Cardiac manifestations Joint involvement Gastrointestinal manifestations Renal manifestations Cutaneous manifestations Inclusion body myositis Malignancies
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INVESTIGATIONS CBC CPK LDH SGPT SGOT Urine C/E Antio myosite specific antibodies ANA RA ENA profile EMG Muscle Biopsy CXR ECG Mammography
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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.
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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.
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Intravenous immunoglobulin (IVIG) Tumor necrosis factor (TNF) inhibitors Rituximab Interferon alfa-2a
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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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