Download Rheumatology and Autoimmune Disease in Emergency Medicine

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
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Degeneration of cartilage
Hypertrophy of bone
Inflammation minimal
Variable hereditary (autoimmune) and
mechanical contributions
Degenerative Arthritis
• Common in:
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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
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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
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APAP
NSAIDS (If failed APAP tx)
Lortab (if acute exacerbation)
Capsaicin cream
Autoimmune (Rheumatoid)
Arthritis
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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
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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
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Pleural effusion
Pericarditis
Lymphadenopathy
Splenomegaly with leukopenia
Vasculitis
RA
• Extraarticular manifestations
– Systemic granulomas
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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
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NSAIDs
ASA
Splints
Heat / Cold
Steroids
Lortab
Crystal Arthritis
• Gout
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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
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Medications (ASA, diuretics, cyclosporine, niacin)
Myeloproliferative disease
Multiple myeloma
Hemoglobinopathy
ES / Chronic RD
Hypothyroidism
Psoriasis
Sarcoidosis
Alcohol use
Gout
• Epidemiology
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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
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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
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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
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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
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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
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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
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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
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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
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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
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RA
SLE
Hypothyroidism
Polymyositis
PMR
Fibromyalgia
• TX
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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
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High fever
Chills
Plunging temp to several degrees below nl
Sore throat
Rash
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Salmon
Nonpruritic
Chest and abdomen
Occurs with fever spike only
Adult Still’s Disease
• S/Sxs
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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
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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
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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
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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
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Aneurysms in medium size vessels
Abdominal pain / abdominal angina
Ileus
Peritonitis
Perforation
SLE
• Why in ED
– Neurologic complications
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Psychosis
Organic brain syndrome
Seizures
Peripheral / cranial neuropathy
Transverse myelitis
Stroke
SLE
• Why in ED
– Glomerulonephritis
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Mesangial
Focal proliferative
Diffuse proliferative
Membranous
– Interstitial nephritis
SLE
• Why in ED
– Miscellaneous
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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
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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
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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
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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
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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