Download Vasculitises`

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.
How does this affect inflammation affect vessels?
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What is common between
Vasculitides?
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Fever
night sweats
Malaise
Myalgia
arthralgia
Savage, C O S et al. BMJ 2000;320:1325-1328
Copyright ©2000 BMJ Publishing Group Ltd.
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
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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
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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
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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
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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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TIFF (Uncompressed) decompressor
are needed to see this picture.