Jonnathan Busko MD EMT-P Albany Medical Center Department of Emergency Medicine Grand Rounds

1. Rheumatology and Autoimmune Disease in Emergency Medicine:The Oxymoron of Rheumatologic Emergencies Jonnathan Busko MD EMT-P Albany Medical Center Department of Emergency Medicine Grand Rounds

2. Why? • A 58 year old male presents to the emergency department complaining of shoulder pain for the last three months. • 10 pound weight loss • Intermittent fevers • Some difficulty getting out of chairs due to stiffness • Comes in today because “I’m tired of it”

3. So… • What does the patient have? • Is this an “emergency?” • Would any other symptom(s) make this an emergency? • What is the treatment?

4. Rheumatology and Autoimmune Diseases in EM • Objectives—by the end of this session, the participant will be able to: • Differentiate between osteoarthridities, rheumatoid arthridities, crystal-induced arthritis, and infectious arthritis • Describe treatment options for patients with painful joints • List admission criteria for patients with painful joints

5. Objectives • Describe the technique for joint aspiration • List and define 2 common pain syndromes seen in the emergency department • Define adult Still’s disease and explain why a patient might present to the ED with it • List 11 criteria for the diagnosis of Systemic Lupus Erythematosus

6. Objectives • List 3 complications of lupus that may bring a patient to the ED • Name and describe the vasculitis most likely to have you say “Call Gus” • Define Giant Cell Arteritis and explain its association with Polymyalgia Rheumatica • List reasons that airway management is critical for patients with relapsing polychondritis

7. Why Are We Discussing This • I saw some of this in medical school • I heard about most of this in medical school • I forgot most of this stuff during my intern year • People now show up with these complaints • I figure I’m not alone here

8. Arthritis (Joint Pain) • Multiple causes for joint pain • 4 important differential diagnoses: • Degenerative arthritis (Osteoarthritis) • Autoimmune arthritis (Rheumatoid arthritis) • Crystal induced arthritis (Gout and pseudo-gout) • Infectious arthritis

9. Degenerative Arthritis • Most common joint disease • 90% > 40 y.o have x-ray changes • Characteristics • Degeneration of cartilage • Hypertrophy of bone • Inflammation minimal • Variable hereditary (autoimmune) and mechanical contributions

10. Degenerative Arthritis • Common in: • Terminal interphalyngeal joints (Heberden’s) • Proximal interphalyngeal joints (Bouchard’s) • MCP / CMC thumb joints • Hip • Knee • MTP joints • Cervical / Lumbar spine

11. Degenerative Arthritis • Secondary • Reactive degneration • Intrarticular (inculding rheumatic arthritis) • Extraarticular causes • Differential • Cool joint • Hard joint (not boggy) • No systemic symptoms • Unlikely to be anything else

12. Degenerative Arthritis • Likely to present in ED for: • Pain Control • Treatment • APAP • NSAIDS (If failed APAP tx) • Lortab (if acute exacerbation) • Capsaicin cream

13. Autoimmune (Rheumatoid) Arthritis • Chronic systemic inflammatory disease • Unknown cause • Chiefly affects synovial membranes • Multiple extra-articular manifestations • Prevalence 1-2% • F:M 3:1 • Usual onset 20-40 y.o.

14. RA • Aggressive disease with high morbidity, decreased life span • Generally treated aggressively

15. RA • Clinical Presentation • Highly Variable • Prodromal symptoms • Malaise • Weight loss • Vague periarticular pain and stiffness • Acute onset • Associated with stress

16. RA • Articular findings • Symmetric joint swelling • Occasionally monoarticular early in dz • Stiffness • Warmth • Tenderness • Pain • Morning stiffness / post-inactivity stiffness

17. RA • Distribution • Any joint • Most common • PIP, MCP, wrists • Knees, ankles, toes • Tendon ruptures from cysts • Nerve entrapment syndromes • Eventual deformity

18. RA • Extraarticular findings: • Subcutaneous nodules • Granuloma with central necrosis • Pleural effusion • Pericarditis • Lymphadenopathy • Splenomegaly with leukopenia • Vasculitis

19. RA • Extraarticular manifestations • Systemic granulomas • Myocardium • Endocardium • Heart valves • Visceral pleura • Lungs • Sclera • Dura mater • Spleen • Larynx

20. RA • Emergency Extraarticular Manifestations • Pericarditis / Pleural effusion • Aortitis • Aortic Regurg • Rupture

21. RA • DDx • Rheumatic fever • Migratory, ASA responsive, Erythema Marginatum • Lupus • CNS involvement, characteristic rash • OA • No systemic disease • Gout • Strong hx of monarticular dz • Infectious • Fever chills, positive tap

22. RA • DDx • Lyme • Typically monoarthritis • PMR • Typically proximal muscles

23. RA • Usually present for Pain • NSAIDs • ASA • Splints • Heat / Cold • Steroids • Lortab

24. Crystal Arthritis • Gout • Metabolic disorder of hyperuricemia • Early monoarticular • Progressive  chronic deforming polyarthritis • Primary • Idiopathic increased purine production • Enzyme defects (Lesch-Nyhan syndrome) • Idiopathic diminished uric acid clearance

25. Gout • Secondary • Medications (ASA, diuretics, cyclosporine, niacin) • Myeloproliferative disease • Multiple myeloma • Hemoglobinopathy • ES / Chronic RD • Hypothyroidism • Psoriasis • Sarcoidosis • Alcohol use

26. Gout • Epidemiology • 90% male • > 30 yo • Can have normal uric acid levels • 5-10% have uric acid kidney stones • Many develop progressive renal failure

27. Gout • Signs / Symptoms • Acute monoarthritis / asymmetric polyarthritis • Often nocturnal • MCP joint great toe (podagra) • Feet / ankles / knees more common • Hips / Shoulders uncommon • Increasing intensity over time

28. Gout • Signs / Symptoms • Skin tense / warm / dusky red • Fever (to 39 C) • Tophi if chronic • Continuous pain if chronic

30. Gout • DDx • Cellulitis • History, joint aspiration findings • Pyogenic arthritis • Joint aspiration findings • Pseudogout • Joint aspiration findings • Post-traumatic inflammation • History, joint aspiration findings • Chronic Lead Intoxication • Systemic lead poisoning sxs

31. Gout • Aspiration findings • Negatively birefringent sodium urate crystals

32. Gout • Why in ED? • First time attack • Multiple attacks pain • Establish your dx • Treat arthritis acutely • NSAIDs x 5-10 d (sx resolution) • Colchicine (poorly tolerated) • Corticosteroids (PO or IA) (TAP FIRST!) • Lortab • Bed rest

33. Chondrocalcenosis / Pseudogout • Chondrocalcenosis • Calcium pyrophosphate dihydrate • Deposited in articular cartilage • Associated with many diseases including true and pseudo-gout • When associated with pseudogout, called: • Calcium pyrophosphate dihydrate deposition disease

34. Pseudogout • Calcium pyrophosphate in joints • Epidemilogy • Age > 60 • Acute, recurrent • Rarely chronic • Most commonly knees and wrists • Also affects • MCP, shoulders, hips, elbows, ankles

35. Pseudogout • DX by hx, joint aspiration • Positively birefringent crystals

36. Pseudogout • Come to the ED for pain. • NSAIDs • Joint aspiration if extensive effusion • IA steroids

37. Pyogenic Arthritis • Intrarticular infection • Nongonococcal, gonococcal, and viral • Nongonococcal • Abnormal host (joint damage, IVDA, endocarditis) • Acute monoarthritis of weight bearing joint or wrist • Large effusions • Causative organism found elsewhere on body

38. Nongonococcal Arthritis • S. aureus most common • Gram – increasing frequency • E. Coli, Pseudomonas • 5-10% mortality • Fever / chills • Joint aspirate > 50K wbc / µL, > 90% PMNs

39. NGA • Abx • Surgical washout vs serial aspirations • Pain control

40. Gonococcal Arthritis • Disseminated Gonococcal disease • Epidemiology • Otherwise normal host • Most common urban pyogenic arthritis • 2-3 x more common in females (esp menses / preg) • Rare at age > 40 • Often identifiable source (cervicitis, urethritis, pharyngitis, proctitis)

41. Gonococcal Arthritis • Signs / sxs • 1-4 day migratory polyarthralgias • Wrists, knees, elbows, ankles • 60% develop tenosynovitis • 40% develop purulent monoarthritis (usually knee) • Characteristic asymptomatic skin rash (most pts) • 2-10 small necrotic pustules • Over extremities, particularly palms / soles

42. GA • DX: • Joint Aspiration • > 50K wbc / µL, • GS + 25% • Culture + 40-50% • CBC—elevated in 30% • Blood cultures • Positive in tenosynovitis 40% • Positive in arthritis 0% • Swab everywhere

43. GA • Treatment • Admit • IV abx • Rocephin / Cefotaxime / Spectinomycin IM

44. Viral Arthritis • Common with systemic viral infections • Short duration • Self-limited • Look for viral cause • Think of HIV / HEP B & C risk factors

45. Joint Aspiration • Prep the site

46. Joint Aspiration • Find your landmarks

47. Joint Aspiration • Enter the joint while aspirating

48. Joint Aspiration • Withdraw as much fluid as possible

49. Pain Syndromes • 2 common pain syndromes • Fibromyalgia • Reflex Sympathetic Dystrophy

50. Fibromyalgia • Chronic widespread musculoskeletal pain syndrome • “Trigger points” • Epidemiology • 3-10% of population • More common in women • Age 20-50 • Similar to CFS except pain prominent feature