Lita Dr Emergency Clinical Rheum a to Logic

RHEUMATOLOGY EMERGENCY IN CLINICAL PRACTICE

LITA DIAH R, DIV. REUMATOLOGI, DEPT PENY DALAM,

RSUD DR SOETOMO SURABAYA

Why?

Approximately 10% to 25% of patients with rheumatologic disorders visiting emergency department require hospital admission one third of the hospitalized patients need intensive care

RA, scleroderma, SLE, 75% ( ICU). 50% ofadmissions infections, 25% - 35% exacerbationof the rheumatologic disorder.

Causes of Life Threatening

. Exacerbation (flare-up) Infections resulting from immunosuppressionAdverse effects of drugs used to treat autoimmune Malignancy resulting from prolonged use of cytotoxic

drugsAcute serious illnesses that are unrelated to the

rheumatic disease

ICU admission

The acute problems leading to ICU admission :gastrointestinal bleeding, cardiac conditions,Pneumoniasepsis interstitial lung disease seizurescerebral hemorrhagePancreatitispulmonaryembolismcerebral infarction

Classification of RheumatologicalEmergencies

rheumatological emergencies can be divided into 2 broadcategories :A. True Rheumatological Emergencies1. Acute low backache2. Acute gout3. Acute arthritis4. Lupus flare5. Systemic necrotizing vasculitides6. Scleroderma renal crisis7. Catastrophic antiphospholipid syndrome8. Erythema nodosum

Classification of RheumatologicalEmergencies

B. Medical Emergencies in Patients withSystemic Rheumatic Disease

– NSAID induced gastrointestinal bleed– Acute left ventricular failure (LVF) in lupus nephritis with hypertension– Intracranial bleed in lupus nephritis with HT– Tuberculous meningitis in SLE– Acute adrenal insufficiency due to sudden steroid withdrawal– Seizures in SLE- Cyclophosphamide induced haemorrhagic cystitis- Drug (immunosuppressive) induced bone marrow suppression, etc.

ACUTE ARTHRITIS

A Acute Monoarthritis– Septic arthritis– Gout– TraumaB Acute Oligo/Poly Arthritis– Reactive arthritis/Reiter syndrome– Viral arthritis– Rheumatic fever– HIV– Disseminated gonococcal infection

ACUTE MONOARTHRITIS

Acute monoarthritis should be considered amedical emergency. The condition warrantsimmediate joint aspiration. Synovial fluid shouldbe aspirated to rule out pus in the joint and crystal identification should be performed

Total and differential WBC counts, culture,Gram’s stain, ZN (Ziehl Neelsen’s) stain andcrystal identification should be performed onall fluids.

Acute gout

Acute gout is one the commonest rheumatologicalemergencies. usually a male, acute pain in one of the lower limb joints. monoarthritis(single joint) or oligoarthritis (2-4 joints).Polyarticular is very rare The diagnosis is made on clinical grounds.Confirmation is by crystal identification aftersynoviocentesis. Serum uric acid levels may benormal. Joint aspiration is mandatory

Gout

Aspiration findingsNegatively birefringent sodium urate crystals

Gout

Why in ED?First time attackMultiple attacks pain

Establish your dxTreat arthritis acutely

NSAIDs x 5-10 d (sx resolution)Colchicine (poorly tolerated)Corticosteroids Bed rest

Pyogenic Arthritis

Intrarticular infectionNongonococcal, gonococcal, and viral

NongonococcalAbnormal host (joint damage, IVDA, endocarditis)Acute monoarthritis of weight bearing joint or wristLarge effusionsCausative organism found elsewhere on body

Non gonococcal Arthritis

S. aureus most commonGram – increasing frequency

E. Coli, Pseudomonas

5-10% mortalityFever / chillsJoint aspirate> 50K wbc / µL, > 90% PMNs

Non gonococcal arthritis

Joint aspiration Joint drainage Surgical arthrotomy for septic hips/shoulders, if osteomyelitis co-exists with septicarthroscopic drainage.Antibiotics need to be given for 2 weeks

parenterally followed by 2-6 weeks of oral therapy.

Gonococcal Arthritis

Disseminated Gonococcal diseaseEpidemiology

Otherwise normal hostMost common urban pyogenic arthritis 2-3 x more common in females (esp menses / preg)Rare at age > 40Often identifiable source (cervicitis, urethritis, pharyngitis, proctitis)

Gonococcal Arthritis

1-4 day migratory polyarthralgiasWrists, knees, elbows, ankles60% develop tenosynovitis40% develop purulent monoarthritis (usually knee)Characteristic asymptomatic skin rash (most pts)

Joint AspirationBlood culturesSwab everywhere

Joint Aspiration

Enter the joint while aspirating

Joint Aspiration

Withdraw as much fluid as possible

Systemic Lupus Erythematosus

Inflammatory Autoimmune SyndromeClinical manifestations from

Trapping of antigen-antigody complexes in capillaries of visceral structuresAutoantibody mediated host cell destruction

Lupus flaremay be precipitated by stress,exposure to sunlight, steroid reduction,pregnancy, infection etc,.

SLE

Why in ED?Ocular

ConjunctivitisBlindness

PulmonaryPleurisyPleural effusionBronchopneumoniaPneumonitis

SLE

Why in EDCardiac

CHFMyocarditsHypertension

Cardiac arrhythmiasVerrucous endocarditis

Valvular incompetenceEmboli

PericarditisMI (Late, 2° chronic steroids)

SLE

Why in EDMesenteric vasculitis

Aneurysms in medium size vesselsAbdominal pain / abdominal anginaIleusPeritonitisPerforation

SLE

Why in EDNeurologic complications

PsychosisOrganic brain syndromeSeizuresPeripheral / cranial neuropathyTransverse myelitisStroke

SLE

Why in EDGlomerulonephritis

MesangialFocal proliferativeDiffuse proliferativeMembranous

Interstitial nephritis

SLE

Why in EDMiscellaneous

Arterial / Venous thrombosisHashimoto’s thyroiditisHemolytic anemiaThrombocytopenia purpuraArthritic pain

SLE

ruled out in febrile lupus patient. A low TLC and normal CRP lupus activity while leukocytosis and raised CRPsuggest infection.

Institution of aggressive therapy beginning with highdose of glucocorticosteroids Infections must be carefully excluded before instituting orincreasing GCS therapy. Consideration of co-morbid disease hypertension, DM, osteoporosis

Management

Initial therapy high dose daily GCS (1-1.5mg/kg) given in divided doses or IV methyl prednisolonepulses (500-1000 mg/d for 3 days) followed by oralprednisolone.Combining with cytotoxic drug is superior to GCS alonein controlling acute severe SLENew therapies for aggressive SLE : intravenousgammaglobulinsB-lymphocyte depletion by rituximab are showing promisingresults in SLE.

Emergent Rheumatologic Complications

Airway obstructionRelapsing poychondritis

Cartilage inflammation / breakdownAirway involved in 50%

RACricoarytenoid dysfunctionCan freeze in closed position

Emergent Rheumatologic Complications

Ventilatory failureDermatomyositis / polymyositis

Muscle failure late in disease

Pleursy / Pleural effusionsRA / SLE

Pulmonary hemorrhageGoodpasture’s, SLE, hpersensitivity vasculitis, SLE, Wegener’s granulomatosis

Emergent Rheumatologic Complications

Pulmonary FibrosisAnkylosing spondolitis, scleroderma, RA

Interstitial pneumonitisMyositis

Admit to r/o infectionImmunosuppress

Emergent Rheumatologic Complications

CardiacPericarditis

RA, JRA, SLE (with other flare sxs)Atherosclerosis

SLEMI

PAN, KawasakiPancarditis

Acute Rheumatic Fever

Emergent Rheumatologic Complications

CardiacValvular heart disease

Seronegative spondyloarthropathiesRelapsing polychondritis

Emergent Rheumatologic Complications

Adrenal InsufficiencyAny rheumatic dz pt on chronic steroidsNo harm in stress doseIf unclear (nonspecific sxs, steroids in past 18 mo)

Cortisol levelDexamethasone

Emergent Rheumatologic Complications

High Morbidity ComplicationsC-spine / Spinal Cord

RA, ankylosing spondylitisTransverse myelitis

SLE

Anterior spinal artery syndrome

Emergent Rheumatologic Complications

High Morbidity ComplicationsBlindness

TASjogren’s Syndrome

RAIndependently

Red Eye in RAEpiscleritis—Painless, self limitedScleritis—Ocular tenderness, blindness, rupture

Emergent Rheumatologic Complications

High Morbidity ComplicationsHypertension

PAN, SLE, RAScleroderma

Was leading cause of deathACEI changed this

Drug induced nephrotoxicity

Emergent Rheumatologic Complications

High Morbidity ComplicationsRenal Disease

GlomerulonephritisSLEWegener’s

Renal vein thrombosisATIII deficiency in SLE / nephrotic syndrome

Microangiopathic disease Diffuse scleroderma—rapidly progressive

Emergent Rheumatologic Complications

High Morbidity ComplicationsRhabdomyolisis

Acute polymyositisMetabolic muscle disease

Management

laboratory testsCRP, ferritin,ANA , CAM, and cytokines .Complement. Serum CRP and PCT used to differentiat exacerbation and infection. Procalcitonin are greatly elevated in acute bacterial and fungal infection butnormal or only mildly elevated in viral inf and flaresCT scan, bronchoscopy, culture sample

Management

Aggressive Corticosteroids.but not effective scleroderma, Kawasaki,HSP, Still’s dis cytotoxic drug ,necrotizing vasculitis, Wegener’s granulomatosis,Goodpasture’s, NL, severe polymyositis,Plasmapheresis NPSLE, hemophagocytic syndrome,Goodpasture’s syndrome,,JRA, TTP, catastrophic APS IvIg dermatomyositis, Kawasaki, ITP, severe NLACE inh :scleroderma renal crisis

Prognosis

Simplified Acute Physiology Score II (SAPS II) scores, poor health status before admission, and cs treatment : poorICU outcome .Thong et al: duration of rheumatic disease ,high doses of CS or immunosuppressiv ~ poor outcome .Mortality is high ;renal failure , coma , ARDS,infectionoverall ICU mortality rate in patients systemic rheumatic diseases 30% to 60% (APACHE) II or SAPS II scores

Terima Kasih