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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 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” So… • What does the patient have? • Is this an “emergency?” • Would any other symptom(s) make this an emergency? • What is the treatment? 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 • 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 • 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 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 Arthritis (Joint Pain) • Multiple causes for joint pain • 4 important differential diagnoses: – Degenerative arthritis (Osteoarthritis) – Autoimmune arthritis (Rheumatoid arthritis) – Crystal induced arthritis (Gout and pseudogout) – Infectious arthritis 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 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 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 Degenerative Arthritis • Likely to present in ED for: – Pain Control • Treatment – – – – APAP NSAIDS (If failed APAP tx) Lortab (if acute exacerbation) Capsaicin cream 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. RA • Aggressive disease with high morbidity, decreased life span • Generally treated aggressively RA • Clinical Presentation – Highly Variable – Prodromal symptoms • Malaise • Weight loss • Vague periarticular pain and stiffness – Acute onset • Associated with stress RA • Articular findings – Symmetric joint swelling • Occasionally monoarticular early in dz – – – – – Stiffness Warmth Tenderness Pain Morning stiffness / post-inactivity stiffness RA • Distribution – Any joint – Most common • PIP, MCP, wrists • Knees, ankles, toes • Tendon ruptures from cysts • Nerve entrapment syndromes • Eventual deformity RA • Extraarticular findings: – Subcutaneous nodules • Granuloma with central necrosis – – – – – Pleural effusion Pericarditis Lymphadenopathy Splenomegaly with leukopenia Vasculitis RA • Extraarticular manifestations – Systemic granulomas • • • • • • • • • Myocardium Endocardium Heart valves Visceral pleura Lungs Sclera Dura mater Spleen Larynx RA • Emergency Extraarticular Manifestations – Pericarditis / Pleural effusion – Aortitis • Aortic Regurg • Rupture 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 RA • DDx – Lyme • Typically monoarthritis – PMR • Typically proximal muscles RA • Usually present for Pain – – – – – – NSAIDs ASA Splints Heat / Cold Steroids Lortab 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 Gout • Secondary – – – – – – – – – Medications (ASA, diuretics, cyclosporine, niacin) Myeloproliferative disease Multiple myeloma Hemoglobinopathy ES / Chronic RD Hypothyroidism Psoriasis Sarcoidosis Alcohol use Gout • Epidemiology – – – – – 90% male > 30 yo Can have normal uric acid levels 5-10% have uric acid kidney stones Many develop progressive renal failure 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 Gout • Signs / Symptoms – – – – Skin tense / warm / dusky red Fever (to 39 C) Tophi if chronic Continuous pain if chronic 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 Gout • Aspiration findings – Negatively birefringent sodium urate crystals 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 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 Pseudogout • Calcium pyrophosphate in joints • Epidemilogy – – – – – Age > 60 Acute, recurrent Rarely chronic Most commonly knees and wrists Also affects • MCP, shoulders, hips, elbows, ankles Pseudogout • DX by hx, joint aspiration • Positively birefringent crystals Pseudogout • Come to the ED for pain. – NSAIDs – Joint aspiration if extensive effusion – IA steroids 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 Nongonococcal Arthritis • S. aureus most common • Gram – increasing frequency – E. Coli, Pseudomonas • 5-10% mortality • Fever / chills • Joint aspirate > 50K wbc / µL, > 90% PMNs NGA • Abx • Surgical washout vs serial aspirations • Pain control 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) 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 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 GA • Treatment – Admit – IV abx • Rocephin / Cefotaxime / Spectinomycin IM Viral Arthritis • • • • Common with systemic viral infections Short duration Self-limited Look for viral cause – Think of HIV / HEP B & C risk factors Joint Aspiration • Prep the site Joint Aspiration • Find your landmarks Joint Aspiration • Enter the joint while aspirating Joint Aspiration • Withdraw as much fluid as possible Pain Syndromes • 2 common pain syndromes – Fibromyalgia – Reflex Sympathetic Dystrophy 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 Fibromyalgia • Signs / Sxs – Chronic pain – Stiffness • Neck, shoulder, low back, and hip pain – General, nonspecific symptoms • • • • • Fatigue Sleep disorders Subjective numbness Chronic HA Irritable bowel syndrome Fibromyalgia • PE – Trigger points • Trapezius • Medial fat pad of the knee • Lateral epicondyle of the elbow Fibromyalgia • DDx – – – – – RA SLE Hypothyroidism Polymyositis PMR Fibromyalgia • TX – – – – – – – Patient education Amitriptyline Fluoxetine Chlorpromazne Cyclobenzaprine Exercise Programs No Narcotics Reflex Sympathetic Dystrophy • Syndrome – Extremity pain and swelling – Trophic skin changes • Skin atrophy • Hyperhidrosis – Vasomotor instability – Shoulder hand variant • Post neck / shoulder injury • Post MI RSD • Signs and symptoms – Usually hand – Ipsilateral restricted shoulder motion • Shoulder-hand syndrome – Diffuse swelling – Burning pain RSD • X-rays – Severe generalized osteopenia • Tx – PT – Prednisone – Stellate ganglion block Adult Still’s Disease • Variant of RA – Prominent high spiking fevers • Dramatic • Up to 40°C – Adults in 20’s and 30’s Adult Still’s Disease • S/Sxs – – – – – High fever Chills Plunging temp to several degrees below nl Sore throat Rash • • • • Salmon Nonpruritic Chest and abdomen Occurs with fever spike only Adult Still’s Disease • S/Sxs – – – – Lymphadenopathy Pericardial effusion Progressive joint symptoms (absent early) Anemia / Leukocytosis (>40K) • DX – Exclude other causes of fever – Fever / Sore Throat / Rash Adult Still’s Disease • TX – ASA – NSAIDS – Prednisone Systemic Lupus Erythematosus • Inflammatory Autoimmune Syndrome • Clinical manifestations from – Trapping of antigen-antigody complexes in capillaries of visceral structures – Autoantibody mediated host cell destruction • Mild to fulminant, life threatening dz SLE • Epidemiology – – – – – – 85% female between menarche and menopause 1:1 M:F in childhood / age > 50 1/1000 white women 1/250 black women 25-70% concordance identical twins Mother with SLE • Daughter 1:40 • Son 1:250 SLE • S/Sxs – General / Systemic • Fever, anorexia, malaise, weight loss • Rash – – – – – – Malar in < 50% Discoid Fingertip lesions Periungual erythema Nail fold infarct Splinter hemorrhages SLE • Diagnosis if 4 of 11 criteria 1) Malar Rash 2) Discoid Rash 3) Photosensitivity 4) Oral ulcers 5) Arthritis 6) Serositis SLE • Criteria 7) Renal dz a) > 0.5 g /d proteinuria or b) > 3+ dipstick proteniuria or c) Cellular casts 8) Neurologic disease a) Sz or b) Psychosis w/o other cause SLE • Criteria 9) Hematologic d/o a) Hemolytic anemia or b) Leukopenia (< 4000/µL) or c) Lymphopenia (<1500/µL) or d) Thrombocytopenia (<100K/µL) SLE • Criteria 10) Immunologic abnormalities a) Positive LE cell preparation or b) Antibody to native DNA or c) Antibody to Sm or d) False positive syphilis serology 11) ANA + SLE • Why in ED? – Ocular • Conjunctivitis • Blindness – Pulmonary • • • • Pleurisy Pleural effusion Bronchopneumonia Pneumonitis SLE • Why in ED – Cardiac • CHF – Myocardits – Hypertension • Cardiac arrhythmias • Verrucous endocarditis – Valvular incompetence – Emboli • Pericarditis • MI (Late, 2° chronic steroids) SLE • Why in ED – Mesenteric vasculitis • • • • • Aneurysms in medium size vessels Abdominal pain / abdominal angina Ileus Peritonitis Perforation SLE • Why in ED – Neurologic complications • • • • • • Psychosis Organic brain syndrome Seizures Peripheral / cranial neuropathy Transverse myelitis Stroke SLE • Why in ED – Glomerulonephritis • • • • Mesangial Focal proliferative Diffuse proliferative Membranous – Interstitial nephritis SLE • Why in ED – Miscellaneous • • • • • Arterial / Venous thrombosis Hashimoto’s thyroiditis Hemolytic anemia Thrombocytopenia purpura Arthritic pain SLE • Important DDx – MUST R/O DRUG INDUCED • Many meds – Chlorpromazine, hydralizine, isoniazid, methyldopa, procainamide, quinidine • Major differences – – – – Sex ratio equal (not so important for individual pt) Nephritis / CNS sxs not present Hypocomplimentemia / anti-DNA ab absent Usually gets better when med stopped SLE • TX – Generally prednisone – Specific tx for disease (stroke, perforation, etc) Temporal Arteritis / Polymyalgia Rheumatica • Spectrum of disease – Affects same population – Affects same individuals – Share HLA haplotypes Polymyalgia Rheumatica • Clinical dx – – – – – Pain and stiffness of shoulder/pelvic girdles Fever Malaise Weight loss ADL failure • Putting on coat, brushing hair – No muscle weakness (vs polymyositis) PMR • Labs – ESR elevated • DDx – Multiple myeloma – Other malignancy – Chronic infection (i.e. bacterial endocarditis) PMR • Tx – Prednisone • 10-20 mg / day • Should improve dramatically in < 72 hours Temporal (Giant Cell) Arteritis • Systemic panarteritis – Medium / Lg vessels – 50% also have PMR • Temporal artery frequently (not always) involved TA • Classic symptoms – – – – – Headache Scalp tenderness Visual changes Jaw claudication Throat pain TA • Blindness – Occlusive arteritis of posterior cilliary branch of ophthalmic artery • 40% have non-classic sxs – Dry cough – Mononeuritis multiplex (usually shoulder) – FUO • 15% all FUO in age > 65 • + rigors and sweats TA • Other physical findings – Pulse asymmetry – Aortic regurg murmur – Bruits over clavicles • Lab findings – Nl white count (even with temp > 40° C) – Elevated ESR TA • TX – To prevent blindness – Prednisone 60 mg po QD – F/U for temporal artery biopsy TA • Important “other” stuff – Thoracic aortic aneurysm 17 x more likely in these patients – Typically occur 7 years post dx True Rheumatologic Emergencies • Associated with systemic rheumatologic disease – SLE – Vaculitides • Associated with treatments for rheumatologic disease – Chronic steroids – GI bleeding (NSAIDs) • Neurologic / Muscular disease – Airway / Ventilation disorders Emergent Rheumatologic Complications • Airway obstruction – Relapsing poychondritis • Cartilage inflammation / breakdown • Airway involved in 50% – RA • Cricoarytenoid dysfunction • Can freeze in closed position Emergent Rheumatologic Complications • Ventilatory failure – Dermatomyositis / polymyositis • Muscle failure late in disease – Pleursy / Pleural effusions • RA / SLE – Pulmonary hemorrhage • Goodpasture’s, SLE, hpersensitivity vasculitis, SLE, Wegener’s granulomatosis Emergent Rheumatologic Complications • Pulmonary Fibrosis – Ankylosing spondolitis, scleroderma, RA • Interstitial pneumonitis – Myositis • Admit to r/o infection • Immunosuppress Emergent Rheumatologic Complications • Cardiac – Pericarditis • RA, JRA, SLE (with other flare sxs) – Atherosclerosis • SLE – MI • PAN, Kawasaki – Pancarditis • Acute Rheumatic Fever Emergent Rheumatologic Complications • Cardiac – Valvular heart disease • Seronegative spondyloarthropathies • Relapsing polychondritis Emergent Rheumatologic Complications • Adrenal Insufficiency – Any rheumatic dz pt on chronic steroids – No harm in stress dose – If unclear (nonspecific sxs, steroids in past 18 mo) • Cortisol level • Dexamethasone Emergent Rheumatologic Complications • High Morbidity Complications – C-spine / Spinal Cord • • • • • RA, ankylosing spondylitis Look for subtle signs post trivial injury VBI Lhermitte sign Transverse myelitis – SLE • Anterior spinal artery syndrome Emergent Rheumatologic Complications • High Morbidity Complications – Blindness • TA – Sjogren’s Syndrome • RA • Independently – Red Eye in RA • Episcleritis—Painless, self limited • Scleritis—Ocular tenderness, blindness, rupture Emergent Rheumatologic Complications • High Morbidity Complications – Hypertension • PAN, SLE, RA • Scleroderma – Was leading cause of death – ACEI changed this • Drug induced nephrotoxicity Emergent Rheumatologic Complications • High Morbidity Complications – Renal Disease • Glomerulonephritis – SLE – Wegener’s • Renal vein thrombosis – ATIII deficiency in SLE / nephrotic syndrome • Microangiopathic disease – Diffuse scleroderma—rapidly progressive Emergent Rheumatologic Complications • High Morbidity Complications – Rhabdomyolisis • Acute polymyositis • Metabolic muscle disease