Vasculitis
Wegener Granulomatosis (Granulomatosis w/ Polyangitis)
Features
3 Cs (Nose – Lung – Kidney)
S/S
·       Nose: saddle-nose deformity, Ulcers
·       Upper respiratory: Sinusitis/otitis,
·       Lower respiratory: Lung nodules/cavitation / tracheal narrowing + ulcers
·       Renal: Rapidly progressive GN
·       Skin: Livedo reticularis, nonhealing ulcers
·       A common sign of wegener is chronic rhinitis that does not respond to usual treatment and getting worse.
·       Arthritis occurs in about 60% of the cases.
Dx
·       C-ANCA is never diagnostic for WG.
·       To dx → bx the affected organ and look for granuloma
·       Skin (leukocytoclastic vasculitis)
Tx
·       Corticosteroids
·       Immunomodulators (MTX, cyclophosphamide)
Giant cell arteritis
Systemic
Fever, fatigue, malaise, weight loss
Localized
·       Jaw claudication: Most specific symptom of GCA
·       Polymyalgia Rheumatica
·       Arm claudication: bruits in subclavian or axillary areas
·       Aortic wall thickening or aneurysms
·       CNS: TIAs/stroke, vertigo, hearing loss
Visual
Amaurosis fugax: Transient vision field
Dx
·       Normochromic anemia
·       Elevated ESR & CRP
·       Temporal artery BX
Tx
·       PMR only: Low-dose oral glucocorticoids
·       GCA: Intermediate-to- high-dose oral glucocorticoids
·       GCA with vision loss: high-dose IV glucocorticoids for 3 days
Takayaso Arteritis
RF
 👩🏻 / Asian / Age: 10-40
Sx
·       Constitutional (fever, weight loss)
·       Arterial (claudication, ulcers) in UL
·       Arthralgias/myalgias
PEx
·       BP discrepancies
·       Pulse deficits
·       Arterial bruits
Dx
·       ⤴️ inflammatory markers (ESR, CRP)
·       CXR: Aortic dilation, widened mediastinum
·       CT/MRI: Wall thickening, narrowing of lumen
Tx
Systemic glucocorticoids  
·       Polyarteritis Nodosa
o   polyarteritis nodosa  → Peripheral neuropathies are very common (mononeuritis – foot drop)
o   How to dx PAN? Biopsy from affected organs  → showing medium a. affected + Angiography showing aneurysms 
o   Tx of PAN? corticosteroids + cyclophosphamide 
·       Churg-Strauss
o   PAN + Asthma
o   Churg-strauss → The cardinal manifestations of Churg-Strauss syndrome are asthma, eosinophilia, and lung involvement (for the sake of remembering this syndrome, you may consider this Churg-Strauss as PAN in an asthmatic patient).
o   WITH CHURG-STRAUSS, ONLY TX W/ PREDINIDISONE (no need for cytotoxic)
·       Temporal arteritis
o   Jaw pain when chewing / pain when combing hair → TA
o   How to manage TA? check ⤴️ESR → corticosteroids → Biopsy 
Henoch-Schonlien purpura
·       Skin bx shows vasculitis of dermal capillaries and postcapillary venules with infiltrates of neutrophils and monocytes; in all tissues, immunofluorescence shows IgA deposition in walls of small vessels and smaller amounts of C3, fibrin and IgM
·       Labs are non dx
o   stool ⊕ for occult blood; ⤴️ serum IgA.
·       Tx: Supportive
o   If proteinuria? ACEI–(non-responsive)–> Steroids (they don’t prevent or alter dz course)

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