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RH20 Rheumatology
Vasculitides
Toronto Notes 2019
Signs and Symptoms
• systemic:fatigue,weightloss,weakness,fever,arthralgias
• dermatologic:livedoreticularis,nodules,purpura,eruptions
• renal:renalinsufficiencyleadingtoHTN
• neuro:Mononeuropathymultiplexinbothmotorandsensorynerves • abdo:abdominalpain,mesentericarteritis
Etiology and Pathophysiology
• focalpanmuralnecrotizinginflammatorylesionsinsmallandmedium-sizedarteries
• thrombosis,aneurysm,ordilatationatlesionsitemayoccur
• healedlesionsshowproliferationoffibroustissueandendothelialcellsthatmayleadtoluminal
occlusion
Investigations
• bloodwork:CBC,ESR,Cr,BUN,p-ANCA,hepatitisBserology • imaging: angiography
• arterialbiopsy
Treatment
• prednisone1mg/kg/dPOandcyclophosphamide2mg/kg/dPO • ±anti-viraltherapytoenhanceclearanceofhepatitisBvirus
Large Vessel Vasculitis
GCA/TEMPORAL ARTERITIS
Table 24. Classification Criteria for GCA*
GCA Criteria
Presence of 3 or more criteria yields sensitivity of 94%, specificity of 91%
Criteria
1. Age at onset ≥50
2. New H/A
3. Temporal artery abnormality
4. Elevated ESR
Description
Often temporal
Temporal artery tenderness or decreased pulsations, not due to arteriosclerosis
ESR ≥50 mm/h
Mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells
• mostfrequentvasculitisinNorthAmerica
• patients>50yr;peakincidence70-80yr
• F:M=2:1
• North-Southgradient(predominanceinNorthernEurope/US) • affectsextracranialarteries
Signs and Symptoms
• newonsettemporalH/A±scalptendernessoverlyingtemporalartery
• sudden,painlesslossofvisionand/ordiplopiaduetonarrowingoftheophthalmicorposteriorciliary
arteries (PCA more common); can affect both eyes
• tongueandjawclaudication(paininmusclesofmasticationonprolongedchewing)
• PMR(proximalmyalgia,constitutionalsymptoms,elevatedESR)occursin30%ofpatients
• aorticarchsyndrome(involvementofsubclavianandbrachialbranchesofaortaresultinginpulseless
disease), aortic aneurysm ± rupture are late complications
• constitutionalsymptomsandshoulder/pelvicgirdlepainandstiffness
Investigations
• diagnosismadebyclinicalsuspicion,increasedESR,increasedCRP,temporalarterybiopsy,and possibly U/S or MRI
Treatment
• ifsuspectGCA,immediatelystarthighdoseprednisone1mg/kgPOindivideddosesforapproximately 4 wk, and then tapering prednisone as symptoms resolve; highly effective in treatment and in prevention of blindness and other vascular complications
• considerlowdoseASAtohelpdecreasevisualloss
• ifpresentingwithvisionlossatdiagnosis,startmethylprednisolone1000mgIVfor3daysfollowedby
high dose prednisone 1mg/kg PO in divided doses for 4 wk
Prognosis
• increasedriskofthoracicaorticaneurysmandaorticdissection • yearlyCXR±abdominalU/Sasscreening
5. Abnormal artery biopsy
*Diagnosed if 3 or more of the above 5 criteria present American College of Rheumatology, 1990
Epidemiology
Medical Emergency
If untreated, GCA can lead to permanent blindness in 20-25% of patients
Treat on clinical suspicion