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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% of admissions infections, 25% - 35% exacerbation of the rheumatologic disorder.

Causes of Life Threatening

. Exacerbation (flare-up) Infections resulting from immunosuppression Adverse effects of drugs used to treat autoimmune Malignancy resulting from prolonged use of cytotoxic drugs Acute serious illnesses that are unrelated to the rheumatic disease

ICU admission

The acute problems leading to ICU admission :

gastrointestinal bleeding, cardiac conditions, Pneumonia sepsis interstitial lung disease seizures cerebral hemorrhage Pancreatitis pulmonaryembolism cerebral infarction

Classification of Rheumatological Emergencies

rheumatological categories :

A. True Rheumatological Emergencies

emergencies can be divided into 2 broad

1. Acute low backache

2. Acute gout

3. Acute arthritis

4. Lupus flare

5. Systemic necrotizing vasculitides

6. Scleroderma renal crisis

7. Catastrophic antiphospholipid syndrome

8. Erythema nodosum

Classification of Rheumatological Emergencies

B. Medical Emergencies in Patients with Systemic 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

Trauma

B

Acute Oligo/Poly Arthritis

Reactive arthritis/Reiter syndrome

Viral arthritis

Rheumatic fever

HIV

Disseminated gonococcal infection

ACUTE MONOARTHRITIS

Acute monoarthritis should be considered a medical emergency. The condition warrants immediate joint aspiration. Synovial fluid should be 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 and crystal identification should be performed on all fluids.

Acute gout

Acute gout is one the commonest rheumatological emergencies. 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 after synoviocentesis. Serum uric acid levels may be normal. Joint aspiration is mandatory

Gout

Aspiration findings

Negatively birefringent sodium urate crystals

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 Bed rest

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

Non gonococcal Arthritis

S. aureus most common Gram – increasing frequency

E. Coli, Pseudomonas

5-10% mortality Fever / chills Joint aspirate

> 50K wbc / µL, > 90% PMNs

Non gonococcal arthritis

Joint aspiration Joint drainage Surgical arthrotomy for septic hips/shoulders, if osteomyelitis co-exists with septic arthroscopic drainage. Antibiotics need to be given for 2 weeks parenterally followed by 2-6 weeks of oral therapy.

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

1-4 day migratory polyarthralgias

Wrists, knees, elbows, ankles 60% develop tenosynovitis 40% develop purulent monoarthritis (usually knee) Characteristic asymptomatic skin rash (most pts)

(usually knee) Characteristic asymptomatic skin rash (most pts) Joint Aspiration Blood cultures Swab everywhere

Joint Aspiration Blood cultures Swab everywhere

Joint Aspiration

Enter the joint while aspirating

Joint Aspiration Enter the joint while aspirating

Joint Aspiration

Withdraw as much fluid as possible

Joint Aspiration Withdraw as much fluid as possible

Systemic Lupus Erythematosus

Inflammatory Autoimmune Syndrome Clinical manifestations from

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

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

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

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

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

Management

Initial therapy high dose daily GCS (1-1.5 mg/kg) given in divided doses or IV methyl prednisolone pulses (500-1000 mg/d for 3 days) followed by oral prednisolone. Combining with cytotoxic drug is superior to GCS alone in controlling acute severe SLE New therapies for aggressive SLE : intravenous gammaglobulins B-lymphocyte depletion by rituximab are showing promising results in SLE.

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 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

Management

laboratory tests CRP, 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 flares CT 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 NL ACE inh :scleroderma renal crisis

Prognosis

Simplified Acute Physiology Score II (SAPS II) scores, poor health status before admission, and cs treatment : poor ICU outcome .

Thong et al: duration of rheumatic disease ,high doses of CS or immunosuppressiv ~ poor outcome . Mortality is high ;renal failure , coma , ARDS,infection overall ICU mortality rate in patients systemic rheumatic

diseases

30% to 60% (APACHE) II or SAPS II scores

Terima Kasih

Terima Kasih