Vasculitises

Outline

• Basics

• Small groups

• Review

Definition

• Inflammation of blood vessel walls

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Symptomatology

• The clinical (and pathological) features are variable.

• Depend on the site and type of blood vessels that are affected.

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How does this affect inflammation affect vessels?

What is common between Vasculitides?

• Fever

• night sweats

• Malaise

• Myalgia

• arthralgia

Copyright ©2000 BMJ Publishing Group Ltd.

Savage, C O S et al. BMJ 2000;320:1325-1328

Large Vessel Vasculitis

• Takayasu’s Arteritis– Primarily affects the aorta and it’s branches

• Temporal Arteritis– Large and medium vessels– Most prominently involves the cranial

branches of arteries arising from the aorta

Medium Vessel Vasculitis

• Polyarteritis Nodosa– Systemic vasculitis– Typically affects small and medium vessels

• Kawasaki disease– Large, medium and small– Particularly the coronary arteries

Small Vessel Vasculitis• Churg-Strauss Arteritis

– Medium and small vessels– Classically involves lung and skin

• Wegener Granulomatosis– Medium and small vessels– Venules and Arterioles as well– Usually involves the upper/lower respiratory tract

and kidneys

• Henoch-Schonlein Purpura– Small vessels

Small Group Cases

• Go through your case with your group (5 min)

• Come up with a diagnosis (5 min)

• Quick Research (5-10 mins)– Signs/Symptoms– Labs/DI– Complications

• Present to Group (3-5 mins per group)

Our Focus today

• Wegener

• HSP

• Kawasaki’s

• Churg-Strauss

Wegener Granulomatosis

• Usually caucasian

• Most frequently in 5th generation

• Males and Females equally affected

Symptoms

• Upper Respiratory– Sinusitis, otitis, oral ulcers

• Lower Respiratory– Cough, hemoptysis, dyspnea

• Kidneys– Blood, protein, casts

• Other (ophth., GI, cardiac, neuro)

First Presentation

• Unexplained Constitutional Sx

• Upper airway– Sinusitis present in 1/2 to 2/3

• Lower Airway

• Other symptoms unusual at presentation

Investigations

• Elevated ESR• N. N. anemia, +/- low plts.• Urinalysis: hematuria, active sediment,

proteinuria• C-ANCA (ANA usually neg.)• CXR: nodular densities (LL’s), pleural

effusions, opacities

Treatment

• Cyclophosphamide +/- prednisone

• Methotrexate +/- prednisone

• +/- septra prophylaxis, +/- plasmapheresis

• IVIg, TNF blockers, etc…

Acute Complications

• Infection

• Pulmonary Hemorrhage

• Renal Failure

HSP

• Children represent 90% of cases

• Association with recent URTI and drugs

• Can affect any age

• IgA deposition in organs

Signs/Symptoms

• Palpable purpura

• Hematuria

• Abdominal pain

• Arthralgia

• Bloody diarrhea

• Fever

The Rash

• Begins as a maculopapular erythema

• Coalesces to form ecchymoses, petechiae, and purpura

• Usually symmetric and in gravity dependent areas

Investigations

• CBC, lytes, Cr

• Urinalysis

• Coagulation studies

• Plain radiography

• Ultrasound

• +/- joint aspiration, biopsy, etc.

Complications

• Renal (usually not severe)

Occasionally, • Intussusception, GI bleeding

Rarely,• Neurologic and pulmonary involvement

Treatment

• Disease is self-limiting

• Supportive treatment

• Hospitalization (dehydration, bleeding, other complications)

Adults

• Rarely get intussusception

• More likely to have renal involvement (including ESRD)

Outpatient Monitoring

• Urinalysis and BP q 1-2 weeks for first 2 months

• Then monthly extending to bi-monthly

• Then during well-child visits

Kawasaki Disease

• More than 85% are less than 5 yrs

Signs and Symptoms

• Fever for 5 days or more– Bilateral conjunctival injection– Mucous membrane involvement– Polymorphous rash– Extremity involvement– Cervical adenopathy

Investigations

• CRP/ESR• CBC• Urinalysis• ALT• Albumin

• Cultures

• +/- echocardiography

Complications

• Coronary Artery Aneurysms• Atherosclerosis/MI• Peripheral arterial obstruction (less

common)

• Other: hyponatremia, dehydration, pancreatitis, arthritis, renal involvement

Treatment

• IVIg– Good evidence showing decrease incidence of

CAA (2g/kg IV over 12 hours)

• ASA– Standard practice? (80-100 mg/kg/d PO divided

qid for 2 wk initial)– Lack solid evidence

• Steroids– Evidence in refractory cases– No good studies for initial management

Churg-Strauss

• Usually follows a predictable pattern:– Prodromal Phase:

– Eosinophilic Phase:

– Vasculitic Phase:

Prodrome

• Usually 2nd-3rd decade– Atopic disease– Allergic rhinitis– Asthma

Eosinophilic

• Peripheral infiltration of organs– Especially lungs and GI tract

Vasculitic

• Life threatening

• May be heralded by the onset of constitutional symptoms

Symptoms

• Asthma (95%)

• Allergic Rhinitis– Or recurrent sinusitis, polyosis, etc

• Skin (2/3)– Palpable purpura, erythema, hemorrhagic

lesions, subcutaneous nodules

Investigations

• CBC

• CXR

• biopsy

Complications

• Cardiovascular disease– Pericarditis, CHF, MI

• Neurologic – Mononeuritis

• Renal– Failure rare

• GI– Abdominal pain

Treatment

• Prednisone +/- cyclophosphamide

THP’s

• Know the basic presentations

• Be aware of complications

• Talk with rheumatology

The End

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