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Rheumatologic Emergencies

Rheumatologic Emergencies. RHEUM EMERGENCIES. Life or organ threatening if not recognized within several hours or days Conditions that won’t first present to a Rheumatologist…. Case. 24 yo female with hx of initial left knee swelling, then right knee swelled Also tenosynovitis of wrist

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Rheumatologic Emergencies

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  1. Rheumatologic Emergencies

  2. RHEUM EMERGENCIES • Life or organ threatening if not recognized within several hours or days • Conditions that won’t first present to a Rheumatologist…..

  3. Case • 24 yo female with hx of initial left knee swelling, then right knee swelled • Also tenosynovitis of wrist • Asp knee 53,000 WBC • Gram stain negative

  4. PYOGENIC ARTHRITIS Bacterial joint space infections are far more destructive than viral or fungal…..

  5. INFECTIOUS (PYOGENIC) ARTHRITIS • Assume any monoarticular arthritis is infectious until proven otherwise • Sudden onset and very painful is more suggestive of crystalline dz – bacterial infection peaks over a few days • If a nearby break in skin, or bacteremia, most definitely approach as infectious process • Septic joint carries high morbidity and mortality • Inflammatory arthritis can mimic septic joint!

  6. INFECTIOUS (PYOGENIC) ARTHRITIS • Risk Factors: • Bacteremia • Adjacent osteomyelitis • Soft tissue infection • Recent injection/aspiration • Penetrating trauma – foreign body • Differential: • Crystalline • Mono presentation of a polyarticular disease

  7. INFECTIOUS (PYOGENIC) ARTHRITIS • Approach • ASPIRATE – culture, gram stain, cell count • > 30,000 would be concerning for infection • Don’t let the presence of crystals fool you • Don’t let scans replace aspiration • Films most likely will be normal – early on • Blood cultures

  8. INFECTIOUS (PYOGENIC) ARTHRITIS

  9. INFECTIOUS (PYOGENIC) ARTHRITIS • Treat while cultures are pending • #1 bug - S. aureus • Other bugs include: • H. flu in kids < than 3 yo • Beta strep in neonates • Various other strep in adults • S. epidermidis and GNR in prosthetic joints • Pseudomonas in IVDA • Salmonella in sickle cell disease

  10. Septic Arthritis • Staph aureus: Healthy adults, skin breakdown, damaged or replaced joints • Strep Species: Healthy adults, asplenic • Neisseria gonorrhea: Sexually active young adults, tenosynovitis • Gram Negative: Immunocompromised hosts, GI infections

  11. INFECTIOUS (PYOGENIC) ARTHRITIS • Treatment Recommendations: • MRSA is a problem => vancomycin • Gram pos => nafcillin • GNR => 3rd gen ceph, add an AG if pseudomonas • Nothing on gram stain => ceftriaxone or cefotaxime • Prosthetic joint => vanco + ciprofoxacin

  12. DGI Not truly an “emergency” but a condition you would not want to miss…

  13. DISSEMINATED GONORRHEA • Most common cause of septic arthritis and tenosynovitis in young adults in N.A. • Complicates less than 1% of all GC cases • Often have an asymptomatic mucosal infection • Major risk factor is absence of C5-8 complement (….remember this for boards)

  14. DGI

  15. DISSEMINATED GONORRHEA • Clues: • Migratory or additive acute oligo (<4) arthritis • Tenosynovitis of wrists / ankles / foot • Fever / chills • Females more commonly affected than males and often within 1 week of menses • 2/3 have rash but it may be scant

  16. DGI May have only ONE vesicle or pustule – so look carefully…

  17. DISSEMINATED GONORRHEA

  18. DISSEMINATED GONORRHEA

  19. DISSEMINATED GONORRHEA • Approach: • Less than 25% of synovial cultures are positive • Less than 10% of blood cultures are positive • Skin biopsy culture is hardly ever positive but may be able to see on gram stain • Best yield is to cultures/DNA probe the mucosal site • Remember the concomitant infection (HIV, syphilis, NSU) • Rx with 3rd generation cephalosporin • Often add doxycycline to cover for Chlamydia

  20. Gram Stain of GC

  21. Case • 53 yo female with hx of SLE c/o acute onset of dyspnea and cough • Had previously been stable • Hgb 7.4, PLT 111, • UA 4+ protein, RBC casts • ds-DNA >370 (markedly positive) • ESR 85

  22. PULMONARY-RENAL SYNDROMES • Acute GN and pulmonary hemorrhage: • Goodpasture’s • Wegener’s • SLE • Post-Strep • Churg-Strauss • Microscopic PAN • ANCA-associated vasculitis • APLA Syndrome

  23. DIFFUSE ALVEOLAR HEMORRHAGE SYNDROME • Capillaritis- Wegener’s, Microscopic PAN, HSP, Cryoglobulinemia, Behcet’s, SLE, RA, MCTD, Scleroderma, Polymyositis • Bland Hemorrhage-Goodpasture’s, SLE, Pulmonary hemosiderosis, Mitral stenosis • Diffuse Alveolar Damage- SLE, Cytotoxic drugs, Cocaine, Infections

  24. DIFFUSE ALVEOLAR HEMORRHAGE SYNDROME • Approach: • Bronch – lavage • ANA • ASO/DNase • ANCA (C-WG / P-MPAN) • Anti-GBM • Treatment: • High dose steroids / cyclophosphamide • Plasmapheresis for Goodpasture’s, possibly SLE - UA - HRCT Reminder: ANA and RF are not “screens” for autoimmune disease ANA simply “rules out” SLE if (-)

  25. Case • 35 yo with SLE • New leg paresthesias and increasing bilateral weakness • Unable to urinate • New fever

  26. TRANSVERSE MYELITIS • Clues: • Leg paresthesias and bilateral weakness • Dermatomal sensory loss • Urinary retention and fecal incontinence • Fever/ axial pain • Abnormal CSF • Approach: • MRI – diffuse edema • Pulse steroids 1000 mg Solumedrol IV for 3 days /plasmapheresis /cyclophosphamide - arteritis of spinal cord - APLA, SLE - hematomas/tumor/fx

  27. CAUDA EQUINA SYNDROME • Ankylosing spondylitis • Spinal / epidural anesthesia • Central herniation / epidural abscess • Dysfunction in multiple lumbosacral nerve roots • Clues: • back pain / rectal pain / posterior leg pain • loss of bowel / bladder control or saddle anes • foot drop • Image and decompress – you have 48 hours!

  28. Cauda Equina Syndrome

  29. Case • 18 yo male with fever • Large joint swelling on/off over last week • Recent sore throat • New murmur

  30. What is this rash?

  31. Erythema Marginatumof - ACUTE RHEUMATIC FEVER

  32. ACUTE RHEUMATIC FEVER • Post group A strep, Strep pyogenes • Clinical dz is self-limited; valvular dz is not • Clues: • Migratory, large joint arthritis • Carditis / valvulitis • Erythema marginatum / sub-Q nodules • Fever • Preceding strep infection • CNS involvement (Sydenham’s chorea)

  33. ACUTE RHEUMATIC FEVER

  34. Mneumonic for Jones’ major criteria • J – Joint swelling • ♥ - Carditis – 2002 update – get an echo • N - Nodules • E – Erythema Marginatum rash • S – Syndenham’s Chorea • Minor Criteria - “PREAF” • PR • Elevated ESR/CRP • ASA – DNase going up • Fever

  35. Cell Walls Plasma Membranes Polysaccharides Cell membranes M Protein Myocardium Myocardial membrane Heart Valves Caudate/thalmic neurons Cardiac Myosin ARF- Cross Reactivity Between Strep/Human Tissue

  36. ACUTE RHEUMATIC FEVER • Approach: • EKG (look for prolonged PR) • Echo-heart valves/carditis • CXR • CBC / ESR / ASO / DNase / throat culture • Not rapid strep • Rx with high dose ASA and give PCN prophylaxis monthly for 5 years • Steroids for heart valve involvement

  37. How Long do you treat? • 10 YEARS Or until 40 – if carditis progressing • 10 YEARS or until 21 – if carditis improves • 5 YEARS or until 21 – no carditis ever

  38. Case • 72 yo male • Mild fever and headache • Arthralgia in shoulders and knees • Cannot get out of chair easily over last week • Difficult to eat • ESR 87

  39. GIANT CELL ARTERITIS

  40. GIANT CELL ARTERITIS • Clues: • > 50 yo • NEW headache (not chronic) • Jaw claudication or arm claudication • Sudden visual loss, diplopia • Systemically ill with many markers of systemic inflammation, increased A. Phos, Ferr, ESR • Approach: • TREAT and then biopsy !! • You have 2 weeks to get the biopsy

  41. Treatment • GCA-High dose prednisone 1 mg/kd or standardly 60-100 mg daily. Continue for several weeks and taper as ESR decreases • Treatment for over 2 years, mostly chronically at low doses < 5 mg pred qd • PMR-start with 20 mg prednisone, taper with watching ESR to < 5 mg pred qd

  42. GIANT CELL ARTERITIS

  43. GIANT CELL ARTERITIS

  44. GIANT CELL ARTERITIS

  45. GCA Fundus photograph showing optic atrophy secondary to giant cell arteritis

  46. GCA-PMR SPECTRUM

  47. Navy Trivia • GEEDUNK – • Ice cream, candy, potato chips and other assorted junk food • Or even the place where they can be purchased

  48. Possibilities of origin • In the 1920's a comic strip character named Harold Teen and his friends spent a great amount of time at Pop's candy store. The store's owner called it The Geedunk for reasons never explained. • The Chinese word meaning a place of idleness sounds something like gee dung. • Geedunk is the sound made by a vending machine when it dispenses a soft drink in a cup. • It may be derived from the German word tunk meaning to dip or sop either in gravy or coffee. Dunking was a common practice in days when bread, not always obtained fresh, needed a bit of tunking to soften it. • The ge is a German unaccented prefix denoting repetition. • In time it may have changed from getunk to geedunk.

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