Case Report – A boy with Muscle ache, Muscle swelling, and Acute kidney injury

1. Case Report – A boy with Muscle ache, Muscle swelling, and Acute kidney injury Dr Sandeep kumar Garg MD DM ANAND HOSPITAL Meerut UP

2. Case report

3. Male • 14 years • Rural background • Student • Total duration of illness- 6 wks

4. Boy with swelled up muscle compartments

5. Symptoms • Pt first noticed difficulty with standing up from squatting position • Later arms were affected, difficulty in flexion & lifting objects • Muscle swelling and pain, in all major compartments especially quadriceps and biceps • Gradual onset & progressively increasing to an extent that pt was bed ridden • Inability to swallow • No visual, sensory, bladder or bowel disturbance

6. Physical examination • At admission • Resp rate - 16/min, • Pulse – 84 / min • BP 132/72 mm Hg • Temp 98.4°F • Alert, responsive, & cooperative • Conjunctiva were anicteric, no pallor & non-injected • Oral mucosa - N

7. Physical examination • No lymphadenopathy, Odema (Paedal, sacral or periorbital) • Muscle groups were swollen • No Clubbing, heliotropic rash • Considerable pain, swelling, and tenseness over the quadriceps muscle compartment • Unable to flex at his knee or being able to bear weight • Peripheral pulses were palpable

8. Systemic examination • Lungs were clear to auscultation, & expansion was normal • Heart sounds were normal • Abdominal examination was normal

9. Systemic examination • CNS Exam • No ocular muscle weakness or ptosis • Weak neck flexion • Facial and muscles of mastication weak • Gag +, No muscle wasting, no fasciculations • No sensory loss • Minimal muscle tenderness • Tone normal

10. Systemic examination • Reflexes: Arm reflexes all present and brisk • Knee reflexes +, ankle jerks +,  Plantars • Power • Shoulders 2-3/5 • Elbow flex/ext 2-3/5 • Hand power 4/5 • Hip flex 2/5, Knee flex 2/5 • rest of leg power - normal

11. Investigations • Hb 12.8 mg/dl, TLC 8900, DLC N 76 L 14 GBP NCNC no evidence of haemolysis or parasite • Urea 68 mg/dl, Creatinine 2.4 mg/dl, Na 138, K 5.4 • Glucose 108 mg/dl, Bilirubin 1.2 mg/dl, SGOT 618, SGPT 234, Alkaline Phosphatase 121, Albumin 4.2 mg/dl • Calcium 8.9 mg/dl, Phosphorus 2.9 mg/dl uric acid 8.2 mg/dl • CPK 15050, LDH 1600

14. USG of Thigh- Generalized increased echogencity

15. USG of Thigh - Generalized increased echogencity

16. EMG of Deltoid

17. EMG of vastus lateralis

18. EMG Normal

19. Muscle Biopsy LP

20. Muscle Biopsy Hp

21. Differential diagnosis • Polymyositis • Myasthenia Gravis • Electrolyte disturbances • Metabolic/endocrine myopathies • Muscular dystrophy • Guillain-Barre Syndrome

22. Treatment • Vigorous IV forced diuresis was initiated • Urine was alkalinized • Urine pH > 6.5 with IV NaHCO3 • IV Methylprednisolone 500 mg/day - 3 days • Followed by Oral Prednisone 60 mg/day

23. Treatment • Serial CPK and RFT were measured, which gradually settled • Electrolytes, calcium, and phosphate remained WNL • Blood in the urine was used as a surrogate marker for myoglobin • By 9th of admission the total CPK dropped to 1319 U/L from 15050

24. Boy with normal size muscle compartmentafter 4 wks

25. Discussion

26. Rhabdomyolysis • Clinical course will depends on underlying cause • Early recognition avoids complications • AKI complicates in 15% of cases • Release of myoglobin is implicated in development of ATN • No correlation between CPK and serum or urine myoglobin • Urine alkalinization (pH>6.5) and forced diuresis prevents ATN

27. Rhabdomyolysis • Severe hyperkalemia secondary to the massive muscle injury is a potential complication • Large amounts of phosphates is released from the muscle cell, causing a relative hypocalcaemia • Hepatic dysfunction occurs in approximately 25% of pts

28. Direct injury to muscle Trauma Burns Infections, like influenza & coxsackievirus Toxins such as alcohol Decreased oxygen delivery to muscle Co poisoning Compression syndrome Arterial occlusion Common causes of Rhabdomyolysis

29. Increased energy consumption Extreme exertion Malignant hyperthermia Heat stroke Drugs as cocaine and amphetamines Decreased energy storage and utilization Diabetic ketoacidosis Non-ketotic hyperosmolar coma Myoedema Hypothermia Common causes of Rhabdomyolysis

30. POLYMYOSITIS • Polymyositis is an idiopathic inflammatory myopathy • Characterize by • Symmetric proximal muscle weakness • Elevated skeletal muscle enzyme levels • Characteristic electromyography (EMG) and muscle biopsy findings

31. POLYMYOSITIS • Female : male predominance 2:1 • Association with HLA DR3 and DRw52. • Peak incidence occurs in the 5th decade • One third of cases are associated with various CTD like RA, SLE, SS and MCTD • One tenth associated with malignancy

32. POLYMYOSITIS • Insidious onset - over weeks to mths but rarely acute • Ocular muscles & facial muscle almost never affected • Distal muscles are spared in 75% of pts • Dysphagia occurs in 25% of patients • Muscle tenderness in 25-50% of cases

33. POLYMYOSITIS • Cardiac disturbance in 30%: conduction disturbances, tachyarrhythmias, CMP • Respiratory impairment in 5%, interstitial lung disease in 10% • Systemic symptoms - fever, malaise, weight loss, arthralgias, Raynaud’s phenomenon

34. CLASSIFICATION OF POLYMYOSITIS - DERMATOMYOSITIS • Gp I: Primary Idiopathic PM • Gp II: Primary Idiopathic DM • Gp III: DM or PM associated with neoplasia • Gp IV: Childhood DM or PM associated with vasculitis • Gp V: PM or DM with associated Collagen VD

35. Renal involvement in polymyositis • Previously thought to be uncommon • But renal involvement is seen in - 21.5% • 1st - ATN with AKI due to myoglobulinemia and myoglobulinuria due to acute rhabdomyolysis • 2nd - Chronic glomerulonephritis has been infrequently reported, usually mesangioproliferative glomerulonephritis

36. Treatment Strategy for PM • Step 1: Prednisone (in aggressive cases, combination with another agent listed in steps 2 & 3 may be preferred) • Step 2: IV - Immunoglobulin • Step 3: Immunosuppressant's, such as Azathioprine, Methotrexate, Mycophenolate or Cyclosporine • Step 4: Newer agents (Rituximab, Tacrolimus, Rapamycin)