Systemic Vasculitis Flashcards
Examples of Systemic Vasculitides
Giant Cell Arteritis IgA vasculitis Polyarteritis Nodosa Buergers Kawasaki Takayasu ANCA (GPA, EGPA, MPA) Lupus Rheumatoid Arthritis Sarcoidosis
When should you suspect a Vasculitis
Multisystem involvement
Constitutional symptoms (fever, weight loss)
High ESR or CRP
Small-vessel involvement (kidney, lungs, skin, etc.)
Examples of LARGE Vessel vasculitis (2 total)
Temporal (Giant Cell) Arteritis
Takayasu Arteritis
Examples of MEDIUM Vessel vasculitis (2 total)
Polyarteritis Nodosa
Kawasaki Disease
Examples of SMALL Vessel vasculitis (3 total)
IgA Vasculitis (Henoch-Schonlein)
Cryoglobulinemic
ANCA (GPA, EGPA and MPA)
IgA Vasculitis (Henoch-Schonlein)
Cryoglobulinemic
ANCA (GPA, EGPA and MPA)
PR3; GPA
cytoplasmic
p-ANCA is an antibody targeted against (PR3/MPO) and associated with (GPA/MPA)
MPO; MPA
perinuclear
ANCA is usually associated with (Crohn’s/UC)
UC
Systemic Vasculitis; c-ANCA (PR3) associated; SMALL vessels; involves the Upper and Lower respiratory tract and glomerulonephritis
Granulomatosis with Polyangiitis (GPA)
GPA is usually associated with what organ systems
RESPIRATORY
Kidney (glomerulonephritis)
Signs/Symptoms of GPA
Saddle-nose deformity
Lung nodules/cavitations
Lung hemorrhage
Pauci-immune glomerulonephritis
Systemic Vasculitis; p-ANCA and SMALL vessel associated; prodrome of allergic/asthma symptoms (~5 years), followed by eosinophilia and systemic manifestations
Eosinophilic Granulomatosis with Polyangiitis (EGPA)
EGPA is usually associated with what organ systems
RESPIRATORY (Rhinitis, Asthma, Pulmonary Infiltrates)
CNS (sensorimotor polyneuropathy)
Skin (petechiae/purpura)
Signs/Symptoms of EGPA
Rhinitis Asthma Pulmonary Infiltrates Sensorimotor Polyneuropathy Petechiae Purpura Arhtralgias/Arthritis
How can you differentiate between GPA and EGPA
GPA: Common, c-ANCA, necrotizing granulomas
EGPA: Uncommon, p-ANCA, eosinophilic granulomas, Rhinitis
Systemic Vasculitis; ANCA and SMALL vessel associated; NON-GRANULOMATOUS; can involve the pulmonary-renal system with hemorrhage or nephritis
Microscopic Polyangiitis
How can you tell between a GPA and MPA
GPA: Granulomas
MPA: No Granulomas
Treatments for ANCA Vasculitides (GPA, EGPA, MPA)
HIGH dose steroids
Immunosuppression
Systemic Vasculitis; SMALL vessel involvement; most common in CHILDREN; usually occurs after an Upper Respiratory Tract Infection; shows Purpura, Arthralgias, Hematuria and possible Abdominal pain
IgA (Henoch-Schonlein) Vasculitis
Signs/Symptoms of IgA Vasculitis
Purpura Arthralgias Hematuria Abdominal pain Hematochezia
Systemic Vasculitis; SMALL vessel involvement; involves immunoglobulins (IgM) that precipitate at LOW temperatures and deposit into vessels, causing inflammation and tissue damage; always involves the SKIN and most patients are Rheumatoid Factor positive and low C4; most common cause is chronic Hepatitis C
Cryoglobulinemic Vasculitis
Lab findings for Cryoglobulinemic Vasculitis
Rheumatoid Factor
low C4
Most common chronic infection involved with Cryoglobulinemic Vasculitis
Hep C
Which immunoglobulin is usually associated with Cryoglobulinemia
IgM
Treatment for Cryoglobulinemic Vasculitis
High dose steroids
Rituximab
Plasmapheresis
Systemic Vasculitis; MEDIUM vessel involvement; INSIDIOUS onset, arthralgia/myalgia, renal ANEURYSMS and with NEUROPATHY
Polyarteritis Nodosa
Signs/Symptoms of Polyarteritis Nodosa
NEUROPATHY
Renal aneurysms
Purpura/Livedo
Arthralgias/Myalgias
Which Vasculitis is associated with NEUROPATHY
Polyarteritis Nodosa
Giant Cell Arteritis usually involves (old/young) patients and their (Temporal/Aortic) arteries
Old (>50); Temporal
Takayasu Arteritis usually involves (old/young) patients and their (Temporal/Aortic) arteries
Young (<50); Aortic
Systemic Vasculitis; LARGE vessel involvement; seen in OLDER patients; symptoms of Polymyalgia Rheumatica predates; involves the TEMPORAL artery, so has symptoms of Temporal headache, jaw claudication and VISION LOSS
Giant Cell (Temporal) Arteritis
Signs/Symptoms of Giant Cell (Temporal) Arteritis
Polymyalgia Rheumatica (prodrome) Temporal headache Jaw Claudication Scalp Tenderness VISION LOSS
Most worrisome consequence of Giant Cell Arteritis
Blindness
Treatment for Giant Cell (Temporal) Arteritis
HIGH dose steroids (Prednisone)
Solumedrol (visual compromise)
Systemic Vasculitis; LARGE vessel involvement; seen in YOUNG Asian patients; involves the Aortic Arch vessels; symptoms relate to limb claudication (unequal BPs, Pulses diminished, limb pain)
Takayasu (Pulseless Arch) Arteritis
Signs/Symptoms of Takayasu Arteritis
Unequal limb BPs
Diminished pulses
Limb pain (ischemia)
Treatments for Takayasu (Pulseless Arch) Arteritis
Prednisone Sugery (bypass occluded vessels)
(can also use MTX or Mycophenolate)
Drugs that can induce a Vasculitis (4 total)
Cocaine
Minocycline
PTU
Hydralazine
What diseases can mimic a Vasculitis
Infective Endocarditis Mycotic Aneurysms Fibromuscular Dysplasia Emboli Antiphospholipid Antibody Syndrome