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 pseudo gout) – 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

• Classic symptoms – Headache – Scalp tenderness – Visual changes – Jaw claudication – Throat pain

TA

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