RHEUMATOLOGICAL Emergency

1. RHEUMATOLOGICAL Emergency Dr.Majeed Shekhany 7th Jan 2014

2. CASE 1

3. History 56 year old male with HTN presents with painful swollen L ankle. The pain started abruptly yesterday. The edema has been progressive and initially started in the L great toe. Similar episodes have occurred over past 1 year. They involved the R wrist, R elbow and L knee and always resolved after a few days after taking Motrin.

4. PMH/PSH: htn, angina MEDS/ALL: nkda; on hctz, metoprolol, aspirin SH: neg x2, works in real estate and only drinks with his clients, the family, the neighbors, etc; lives with wife and 2 children FH: htn, cad, renal stones, brother is alcoholic

5. Physical t 101, bp 160/100, wt 210 lbs Exam wnl overall Purple and tender L first MTP joint with erythema and swelling up the foot to the ankle Enlarged bursa on R elbow

7. a. Trauma b. Crystal induced arthritis c. Fungal septic arthritis d. Bacterial septic arthritis e. Rheumatoid arthritis f. Osteoarthritis g. Reiter’s syndrome h. Prodrome of hepatitis B What are the 2 most reasonable choices in the differential diagnosis?

8. a. CBC, SMA 12, UA b. Arthroscopy c. ANA, RF d. ESR/CRP e. Blood cultures f. X-ray the feet g. X-ray the hands h. Aspirate the MTP joint What would be your next step?

9. Labs & Tests WBC 12,200 (85% PMN, 10% Bands), Hct 41% Ast 60, Alt 73, Alk phos 225 Creatinine 1.2, UA wnl L 1st MTP joint arthrocentesis  3 drops of fluid with PMN’s on gram stain but no organisms X ray L foot  soft tissue swelling and bony erosion of 1st MTP joint slightly removed from the joint with overhanging edge

10. Under polarizing microscopy see: Intra- and extracellular needle shaped crystals Yellow when aligned parallel to the slow axis of rotation

11. What is your diagnosis Doctor? More specifically, what does your patient have in common with Michelangelo? Raphael’s fresco at the Vatican “School of Athens” 1509

12. Gout First described 5th century BC by Hippocrates Uric acid overproduction OR underexcretionurate deposition in joints (first MTP joint of great toe) and kidneys with primary and secondary causes Obese alcoholic male with DM, HTN, HL abusing his medications stumbling around like his father with a painful toe Diagnosis by aspiration of joint or can be made clinically

13. Gout Treatment Terminate acute attack nsaids, steroids, +/- colchicine Prevent recurrence xanthine oxidase inhibitor (allopurinol), uricosoruic (probenecid), dietary modifications Prevent complications goal uric acid < 6.4 mg/dl Reverse associated conditions control DM, HTN, etoh abuse, etc

14. CASE 2

15. History 34 yo male with IDDM presents with 3 hours of pain and swelling in the L knee and R shoulder. The pain started after an episode of chills. One day prior he had twisted his L knee and scraped his R elbow.

16. PMH/PSH: juvenile onset DM MEDS/ALL: nkda, on insulin SH: neg x 3, is sexually active, works as a lawyer FH: nc

17. Physical PE: t 102, p 110, Appears ill overall 1x1cm carbuncle on the neck R shoulder ROM limited because of pain with focal tenderness Superficial skin abrasion over R olecranon L knee swollen, red, warm, tender with decreased ROM due to pain but no instability

18. Stat Labs Wbc 20, 000 with 70% PMN, 20% bands Hb 15 PT normal Glucose 350 Creatinine 1.2 UA- glucose 3+

19. a. Rheumatoid arthritis b. Ankylosing spondylitis c. Septic arthritis d. Hemarthrosis of shoulder and knee e. Biceps tendon rupture f. Medial meniscus tear g. Anterior cruciate ligament rupture h. Rotator cuff tear i. Reiter’s syndrome j. Acute gout k. Diabetic neuropathic arthropathy The most reasonable diagnosis are:

20. a. MRI of the knee b. Bone scan c. Arthrocentesis d. Arthroscopy e. X- rays of shoulder and knee f. Blood culture g. Skin abscess culture h. Chest x-ray i. AP pelvis x-ray j. Serum uric acid Which diagnostic tests would you order?

21. Diagnostics X-rays of both joints  normal articular surfaces with effusion and soft tissue swelling evident on knee films Arthrocentesis of both joints  yellow cloudy appearance  80,000 Wbc with 95% PMNs Gram + cocci in clumps and chains

22. Classes of Synovial Fluid

23. What is your diagnosis Doctor? What does your patient have in common with the Summoner in the Canterbury Tales? From the Ellesmere manuscript (15th century) of the Canterbury Tales by Geoffrey Chaucer (14th century)

24. Septic Arthritis Arthritis due to seeding of a joint by a bacteria, mycobacterium or fungus usually via hematogenous spread. Classify as gonococcal and non-gonococcal Adult  G (-) Cocci  50% N. Gon  G (+) Cocci  45% Staph A or Strep Pneumo Prosthetic joints  Staph epi or Staph aureus

25. Conditions That Predispose a Patient to Joint Infections Immune suppression cancer, DM, liver disease, SLE, sickle cell disease, complement deficiency Joint destruction RA, gout, pseudogout, OA, hemarthrosis Regular introduction of organism IVDA, pregnancy, menses, etc

26. Treatment of Septic Arthritis Choose 3 courses of action before the synovial fluid culture and sensitivity test results are available

27. a. Admit to hospital b. Oral penicillin V with bed rest at home c. IV penicillin d. IV vancomycin alone e. Aminoglycoside and 3rd Gen cephalosporin f. IV nafcillin plus aminoglycoside in hospital g. Daily needle aspiration h. Joint lavage i. Surgical debridement and drainage j. IV colchicine k. Intraarticular steroids l. High dose prednisone for 1 week

28. CASE 3

29. History 72 yo male complains of headaches and muscle pains. The headache started 3 months ago when he was involved in a MVA. He has had daily R sided headaches since that are now worsening and most severe in the evening but are present upon awakening. Tylenol does not help. He also has some general malaise, 5 pound weight loss, aching around the hips and shoulders and morning stiffness in the legs.

30. PMH/PSH: htn MEDS/ALL: nkda; on hctz, asa SH: smokes a pipe, occasional etoh use FH: nc

31. a. Hypertensive encephalopathy b. Subdural hematoma c. Brain tumor d. CVA e. Giant cell arteritis g. Migraine h. Rheumatoid arthritis i. Cerebral artery aneurysm j. SLE What is in our differential diagnosis?

32. Further History Elicited Low grade temperature (99-100ºF) He did have an episode of decreased vision in the L eye and pain on the R side of his face when he chews his food. He also states his scalp hurts when he combs his hair.

33. Physical t 37.6, p 100, bp 150/95 R, 145/90 L Fundoscopic exam: arteriolar narrowing without hemorrhage or exudate, optic discs well demarcated. Slight R ptosis with tenderness to palpation along temporal area. Tenderness around the shoulder girdle with no true muscle weakness.

35. a. ESR/CRP b. CBC with diff c. UA d. Serum creatinine e. CPK f. ANA h. Rheumatoid factor i. Spinal fluid analysis j. Chest x ray k. Brain MRI l. Cerebral angiogram Which of the following tests would be abnormal?

36. What is your diagnosis Doctor? What does your patient have in common with this Bishop? Virgin with Canon Van der Paele by Jan Van Eyck 1436

37. Giant Cell Arteritis Chronic, patchy vasculitis of medium and large sized vessels mostly in patients over the age of 50 Most commonly presents with polymyalgia rheumatica in 40% of cases PMR= symmetrical pain/stiffness in shoulder and pelvic girdle muscles for more than 1 month in a patient age > 50 with elevated ESR with rapid response to steroids

38. SYSTEMIC FINDINGS: PMR Fever Anorexia Malaise Weight loss Elevated ESR Abnormal lfts LOCAL MANIFESTATIONS: Temporal headache Blindness Scalp necrosis Jaw claudication Cranial and peripheral neuropathies Rare organ involvement GCA Clinical Findings

39. Diagnosis of GCA Diagnose with 3/5 of the following: Age > 50 years New headache Temporal artery abnormality ESR > 50mm/hr Abnormal artery biopsy with mononuclear cell infiltrate, granulomatous inflammation, usually with multinucleated giant cells

40. a. Anticoagulation b. Oral analgesics c. IV steroids d. Temporal artery biopsy now e. Temporal artery biopsy at later date f. Aspirin h. Referral to neurology i. Oral steroids Now what is your next step?

41. Treatment of GCA Corticosteroids Oral prednisone 1mg/kg/day IV steroids if visual loss, etc Obtain temporal artery biopsy within 7-14 days Follow symptoms and ESR

42. Treatment of GCA (cont’d) Cytotoxic agents if steroid resistant Methotrexate Azathioprine Cyclophosphamide Monitor for late thoracic aortic aneurysms Relapses in 60%

43. Case 5

44. History 36 yo female with 6 month history of Raynaud’s phenomenon. She also complains of pain in the wrists, fingers and ankles. She has daily morning stiffness x 45 minutes and has bilateral hand swelling.

45. PMH/PSH: G1P1001, nsvd x 1, regular menses, h/o appy MEDS/ALL: nkda, on mvi daily SH: smoker, neg x 3, no blood transfusions FH: cousin with SLE

46. Physical bp 110/70, t 37.8, p 88, overall in nad. Edema of hands, fingers, legs from the knees down. Skin thickening of hands extending to the elbows with periungal erythema present No synovitis

48. a. Giant cell arteritis b. Rheumatoid arthritis c. Polymyositis d. Dermatomyositis e. Sjögren’s syndrome f. Scleroderma g. Hepatitis b prodrome h. Ankylosing spondylitis i. Polyarteritis nodosa j. Systemic lupus erythematosus List the most reasonable diagnoses:

49. a. CBC b. BMP c. Liver enzymes d. UA e. ESR/CRP f. RF g. ANA h. Anti-dsDNA i. Anti-SCL-70 j. Anti-centromere Ab k. TSH l. Hand films m. Nailfold capillary microscopy What tests would you choose next?