Vasculitises

Outline Basics Small groups Review

Definition Inflammation of blood vessel walls

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?

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:

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? ( 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