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

8. 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

9. INFECTIOUS (PYOGENIC) ARTHRITIS

10. 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

11. 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

12. 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

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

14. 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)

15. DGI

16. 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

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

18. DISSEMINATED GONORRHEA

20. 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

21. Gram Stain of GC

22. 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

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

24. 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

25. 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 (-)

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

27. 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

29. 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!

30. Cauda Equina Syndrome

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

32. What is this rash?

33. Erythema Marginatum of - ACUTE RHEUMATIC FEVER

34. 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)

35. ACUTE RHEUMATIC FEVER

36. 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

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

38. 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

39. How to Treat GAS pharyngitis

40. 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

41. 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

42. GIANT CELL ARTERITIS

43. 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

44. 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

45. GIANT CELL ARTERITIS

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

49. GCA-PMR SPECTRUM

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

51. 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.

52. Case 64 yo female with 30 year hx of poorly controlled RA Paresthesias in extremites with neck movement Scheduled for cholecystectomy next week From too much Geedunk

53. CERVICAL SUBLUXATION WITH RA

55. Clues: –Long-standing RA with or without neck pain Presentation: –Hyperreflexia / VBI symptoms –Sphincter dysfunction –Sensory findings in extremities –C1-C2 subluxation greater than 9 mm; presence of atlantoaxial impaction, worse prognosis-neurosurgical evaluation. Approach: –Caution in C-spine manipulation –Image (plain films and MRI)

56. Case – Not Hunky-Dori

57. SCLEROMALACIA PERFORANS

58. RA EYE INVOLVEMENT

59. RHEUMATOID ARTHRITIS EYE DISEASE Episcleritis Anterior scleritis with corneal melt Scleromalacia Scleromalacia perforans

60. EPISCLERITIS

61. SCLERITIS / CORNEAL MELT

62. SCLEROMALACIA

63. Case 43 yo female with SLE New mental status changes Fever, nausea Joint Pain Bruising New thrombocytopenia 43 K, Hgb 8.3 Creatinine 3.4

64. AUTOIMMUNE COAG DISORDERS TTP-Neurologic changes, fever, renal failure, thrombocytopenia, microangiopathic hemolytic anemia Plasmapheresis, Pulse steroids DIC, AIHA APS Syndrome –

65. CATASTROPHIC ANTIPHOLPHOLIPID SYNDROME -Gangrene of limb -Recurrent fetal loss -Livedo reticularis -Migraine -Renal insufficiency -PHTN -PE / DVT -Neuro deficits -AVN -Endocarditis -:ow plts – (Sneddon’s syndrome is low plts, stroke, and livedo) Mortality 50% -TX with - anticoagulation/plasmaphesis

66. What is this called?

67. AUTOIMMUNE COAG DISORDER

68. Don’t Confuse with: Cutis Marmorata Telangiectatica Congenita “We believe that cutis marmorata telangiectatica congenita is a relatively mild condition. The prognosis is usually good, with minor associated anomalies. Improvement of the mottled, vascular pattern is usually observed within 2 years. We recommend careful clinical examination of all patients to exclude any associated anomalies. Patients should be referred to a neurologist or an ophthalmologist only if symptoms are present or if vascular lesions are present around the eyes.” Arch Dermatol. 1999;135:34-38

69. Case 43 yo female with scleroderma Recently was given prednisone for joint pain by PCM BP 182/108 Creatinine 4.3 Hgb 8.3 helmet cells on smear

70. SCLERODERMA RENAL CRISIS

71. Silent renal failure Cardiac decompensation HTN (relative) Microangiopathic hemolytic anemia Rx with ACE Inhibitor Captopril in increasing doses Do not stop the ACE

72. SCLERODERMA RENAL CRISIS

75. Case 24 yo male New onset of testicular pain Purpuric rash New foot drop BP 158/108

76. SYSTEMIC VASCULITIS Mononeuritis multiplex Glomerulonephritis Skin infarction / ulceration Neurologic dysfunction Multi-organ infarctions Always think of systemic vasculitis in the setting of multi-organ disease!

77. Major Vasculitidies Polyarteritis Nodosa Microscopic Polyangiitis Churg-Strauss Syndrome Wegener’s Granulomatosis Cryoglobulinemia Start high dose 60-100 mg day Or pulse steroids 1 gram Solumedrol for 3 days Add cyclophosphamide to Wegener’s treatment

78. Case 72 yo with ASCVD 3 days S/P catheterization Complained of blue toes Creatinine 2.7 microhematuria Mild eosinophilia

79. Blue Toes

80. CHOLESTEROL EMBOLIC SYNDROME - Blue toes after an intervention - TIA - ARF - Microhematuria - Fever - Oschemic bowel - Eosinophilia - ESR increased - Heparinization may worsen by releasing clefts from plaques

81. The Dreaded Friday afternoon Consult 35 year old female Tearful in office Total body pain, worst pain of her life Abdominal pain, urinary urgency issues Severe Fatigue Cannot perform ADL’s Has been to several physicians for these complaints

82. Fibromyalgia Fulminans Treatment Empathy from the doctor Detailed explanation of the condition Prescription for slowly increased exercise –Log book –F/u with PCM (that may be you)

83. RHEUMATOLOGIC EMERGENCIES ????? ???