Common Rheumatologic Problems & Urgencies/Emergencies

1. Common Rheumatologic Problems & Urgencies/Emergencies 7/25/2019

2. Case 1

3. CASE 1 • 56 year old male with HTN • CC: pain & swelling of left ankle • Awoke with acute pain & swelling in the left great toe 2 days ago; now similar pain & swelling in the ankle • Similar episodes a year ago involving the right wrist & elbow & left knee • Occur every few months • Resolved previously after a few days of taking high dose ibuprofen • Meds: HCTZ, metoprolol, aspirin • Soc: real estate, a few drinks a week; no tobacco or illicits; lives with wife and 2 children • Fam: HTN, CAD, alcoholism, renal stones

4. PHYSICAL EXAM Vitals: T: 101F, BP: 160/100 • Erythema, warmth, swelling and tenderness of left first MTP joint, the dorsal foot and the left ankle • Enlarged Right olecranon bursa containing small non-tender hardened masses

5. Which are the top 2 diagnoses in the DDX? a. Trauma b. Crystal induced arthritis c. Septic arthritis d. Rheumatoid arthritis e. Osteoarthritis f. Reiter’s syndrome

6. What would you do next? • CBC • Serum uric acid • Arthroscopy • ANA, RF • X-ray the feet • Aspirate the MTP joint

7. Labs 13.6 14.2 250 41 Segs 95, Bands 10

8. Imaging X ray L foot Soft tissue swelling and bony erosion of 1st MTP joint slightly removed from the joint with overhanging edge

9. Arthrocentesis Left 1st MTP joint  3 drops of hazy yellowish fluid

10. Polarizing Microscopy • Intra- and extra-cellular needle shaped negatively bi-refringent crystals • Yellow when aligned parallel to the axis of the polarizer • Urate crystals Gram stain: Moderate WBCs but no organisms Culture: negative

11. Gout • Inflammatory arthritis caused by uric acid crystal precipitation in joints • Uric acid deposition in joints & tissues (kidneys) • Uric acid is end product of degradation of purines • overproduction OR under excretion • History usually very characteristic • Podagra • Definitive diagnosis: demonstration of uric acid crystals in joint fluid (or presence of tophi); otherwise see ACR criteria

12. Gout Treatment • Terminate acute flare • NSAIDS (at anti-inflammatory doses) • Steroid burst/taper • Oral colchicine • Uric acid lowering therapy -Prevent recurrence • Allopurinol, Probenecid, Dietary changes • Start after resolution of acute flare • Goal of uric acid < 6 • Usually require these for life • High risk of flare during titration of uric acid (do NOT stop these when patient is admitted to the hospital)

13. Indications for Chronic therapy of Gout • >2-3 attacks of gout within 1 year • Tophaceous gout • Nephrolithiasis • Chronic gouty arthritis with bony erosions • Asymptomatic hyperuricemia with serum uric acid >12 • Chronic renal impairment

14. Case 2

15. Case 2 • 34 yo male with IDDM • 3 hours of pain and swelling R shoulder • +chills • +fell the day before and twisted his L knee, scraped his R shoulder • PMHx: juvenile onset DM • Meds: insulin • Soc: neg x 3, is sexually active

16. Physical Exam • Vitals: T 102, P 110 • Gen - ill appearing • Skin- skin abrasion with surrounding erythema and warmth over R shoulder • CV-Tachy, RR, no m/r/g • Musculoskeletal-R shoulder with erythema, swelling and limited ROM

17. Labs 15 Segs 75, Bands 20 20 400 45 PT nl Glucose 350 Creatinine 1.2 UA- glucose 3+

18. Top diagnoses in the differential? • Rheumatoid arthritis • Septic arthritis • Hemarthrosis of shoulder and knee • Ligamentous injury of knee • Rotator cuff tear • Reiter’s syndrome • Acute gout

19. Which diagnostic tests would you order next? a. MRI of the knee b. Arthrocentesis c. Arthroscopy d. X- rays of shoulder f. Blood cultures g. AP pelvis x-ray h. Serum uric acid

20. Diagnostics • X-rays  normal articular surfaces with effusion and soft tissue swelling • Arthrocentesis  yellow cloudy synovial fluid Cell count-WBC 80,000, Segs 95% Gram stain: Gram + cocci in clusters Culture: Staph aureus

21. Synovial Fluid Analysis • Normal • Clear, viscous, pale yellow • 0-200 WBC, <10% Segs • Non-inflammatory • Clear to slightly turbid • 200-2000 WBCs, <20% Segs • Inflammatory • Slightly turbid • 2000-50,000, 20-70% Segs • Pyarthrosis • Turbid • >50,000 WBCs, >70% Segs

22. Septic Arthritis • Arthritis due to seeding of a joint by a bacteria, mycobacterium or fungus • Classified as gonococcal or non-gonococcal • Non-gonococcal arthritis is the more potentially destructive form • Staph aureus is the most common bacterial etiology in adults in both native and prosthetic joints • S. epidermidis and anaerobic organisms are common in prosthetic joints • GNR in elderly and immunocompromised

23. Gonococcal vs Non-gonococcal Gonococcal • Young, healthy adults • Migratory polyarthralgias/arthritis • Tenosynovitis common • Rare positive Bld cx • Positive joint cultures <25% Non-gonococcal • Young children, elderly, immunocompromised • Monoarthritis • Tenosynovitis rare • Positive Bld cx 40-50% • Positive joint cultures >95%

24. Mechanisms of Joint infection • Most common-hematogenous spread from remote infection-(70%) • Spread from adjacent osteomyelitis • Lymphatic spread from infection near the joint • Iatrogenic infections from arthrocentesis or arthroscopy (20%) • Penetrating trauma from contaminated objects • Predisposing conditions • Immune suppression • Underlying arthritis or trauma • Regular introduction of organism

25. Septic Arthritis Therapy • Antibiotics ASAP after tap • If Gram stain reveals GPC. Cefazolin for community acquired infections & vancomycin for hospital or nursing home acquired infections • If Gram stain reveals GNR-use 3rd generation cephalosporin (ceftazidime, ceftriaxone). Add an aminoglycoside like gentamicin if Pseudomonas is likely (IVDA) • +/- Joint drainage: serial taps, arthroscopic lavage, surgical debridement and drainage • Admit to the hospital with IV ABX

26. CASE 3

27. Case 3 • 72 yo male w/ HTN • CC: headaches and shoulder pain • Constant aching of shoulders & hips with morning stiffness. • Tylenol does not help • 3 months ago, was in MVA with daily R sided HA since then. The headache is now worsening, most severe at night but is present upon awakening & is constant. • He also reports general malaise, a 5 pound weight loss, and low grade temperature (99-100ºF) • Episode of transient decreased vision in the L eye a few days ago that has improved • PMH: HTN • MEDS: HCTZ, ASA • SH: smokes a pipe, occasional ETOH use • ROS: c/o pain on the right side of his face when he chews his food; Scalp also hurts when he combs his hair

28. Physical Exam • Vitals: T 99.6F, P 100, BP 150/95 R, 145/90 L • Fundoscopic exam: arteriolar narrowing without hemorrhage or exudate, optic discs well demarcated • tenderness to palpation along R temporal area • Pain with movement of the shoulder and hip girdle with no true muscle weakness

29. Differential diagnosis? a. Hypertensive encephalopathy b. Subdural hematoma c. Brain tumor d. CVA e. Giant cell arteritis g. Migraine h. Cerebral artery aneurysm i. Rheumatoid arthritis j. SLE

30. Giant Cell Arteritis • Large Vessel Vasculitis • Patients generally over the age of 50; average age is 70 • Multinucleated giant cells infiltrate vessels leading to necrosis, thrombosis; patchy involvement • Sx of 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

31. GCA Clinical Findings SYSTEMIC: • PMR • Fever • Anorexia • Malaise • Weight loss • Elevated ESR LOCAL: • Temporal headache • Visual disturbances/Blindness • Scalp tenderness/necrosis • Jaw claudication • Cranial and peripheral neuropathies

32. 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

33. Treatment of GCA • Corticosteroids Oral prednisone 1mg/kg/day IV steroids if impending visual loss • Obtain temporal artery biopsy within 14 days of starting steroids • Follow symptoms and ESR • Cytotoxic agents if steroid resistant Methotrexate Tocilizumab (Actemra) Monitor for late thoracic aortic aneurysms • Relapses in 60%

34. Case 4

35. Case 4 • 34 y/o healthy female • CC: 6 month history of pain and color change in her fingers with cold exposure • Fingers turn white and blue even in air conditioning. • Hand swelling bilaterally & pain in wrists, fingers & ankles • Daily morning stiffness x 45 minutes • No muscle weakness • PMH/PSH: G1P1, h/o appendectomy • Meds: MVT • SH: neg x 3, no blood transfusions • FH: cousin with SLE

36. Physical Exam • Vitals: T 37.8, BP 110/70, P 88 • Gen- NAD • Edema of hands diffusely with skin thickening extending to the elbows bilaterally and same in legs from the knees down • Periungual erythema present • No synovitis

37. Most reasonable diagnosis? • Giant Cell Arteritis • Rheumatoid arthritis • Polymyositis • Dermatomyositis • Sjogren’s syndrome • Ankylosing spondylitis • Primary Raynaud’s • Scleroderma • Systemic lupus erythematosus

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

39. Labs 13.1 7.8 265 40.2 Creatinine 0.9 UA nl ANA >1:1280; nucleolar Anti centromere neg Anti dsDNA neg Anti SCL 70 positive

40. Scleroderma • Heterogeneous group of disorders with thickened skin as a hallmark • Endothelial disruption and fibroblast proliferation with unknown etiology • Categorized into localized and systemic based on extent of skin involvement

41. Localized Scleroderma • Affecting cutaneous and subcutaneous tissues only • Morphea- one or more patches of sclerotic skin on trunk and limbs • Linear- a line of thickened skin affecting one or more extremities, can be dermatomal • Scleroderma en coup de sabre – subset of linear disease affecting the forehead and face

42. Systemic Sclerosis • Skin and internal organ involvement • Limited/CREST • Skin involvement limited to hands, face/neck • pulmonary HTN, GI involvement • Anti-centromere antibody • Diffuse • Extends proximal to MCPs, involves trunk • ILD, CV disease, renal disease, joint deformities, myopathy • Anti-Scl-70

43. Raynaud’s

44. Sclerodactyly • Early • Late • Picture Courtesy of Martin Pattrick

45. Periungual Erythema

46. Nailfold Capillaroscopy

47. Scleroderma Diagnosis and Treatment • Diagnosis = clinical sclerosis +/- organ involvement & autoantibodies • Treatment is symptomatic • ARBs, Calcium channel blockers, Viagra • Proton pump inhibitors • Manage pulmonary, CV issues

48. Back to our patient…3 months later… • Pt c/o SOB and malaise • VS - BP 150/90, tachy, tachypneic • HEENT - distended neck veins • Pulm-bilat crackles at bases • Skin -thickening has progressed to mid upper arm • Labs: • Hgb 1310, Plt 26585,000 • Microangiopathic changes noted on peripheral smear • Creatinine 0.93.6 • UA: 1+ protein, 5-10 RBCs

49. Our patient • Admitted to ICU • Intubated for respiratory failure • What is your diagnosis now??

50. Scleroderma Renal Crisis • Severe, life-threatening complication of scleroderma • 10 to 15 % of scleroderma patients • More frequent in patients with diffuse rather than limited scleroderma • Often associated with RNA polymerase III antibody