Vasculitis Flashcards
Clinical Presentation:
Older adults with hypertension
Younger individuals with Marfan syndrome or Ehlers-Danlos syndrome
Severe, tearing chest pain radiating to the back
Can have decreased carotid pulses or decreased/loss of extremity pulses
Pertinent Lab Findings:
Imaging shows intimal tear and blood dissecting the media
Buzz Words:
Cystic medial necrosis
Hyaline arteriolosclerosis of vasa vasorum
Proximal 10 cm of aorta
Pericardial tamponade (most common cause of death)
Aortic Dissection
Types:
True aneurysm (all layers intact)
False aneurysm (pseudoaneurysm) (defect in wall, extravascular hematoma)
Dissection (blood enters arterial wall)
Pertinent Lab Findings:
Imaging: Fusiform/saccular dilation
Buzz Words:
Balloon-like dilation
Tree-bark aorta (tertiary syphilis)
Pulsatile abdominal mass
Thrombosis with embolism
Aneurysms
Clinical Presentation:
Tall, long limbs, arachnodactyly
Pectus excavatum
Aortic root dilation, aortic dissection
Pertinent Lab Findings:
Defect in fibrillin-1 (connective tissue)
Buzz Words:
Positive thumb sign
Cystic medial necrosis
Lens dislocation (upward)
Aortic aneurysm/dissection
Marfan Syndrome
Clinical Presentation:
Hyperextensible skin, hypermobile joints
Easy bruising, fragile tissue
Aortic aneurysm/dissection
Pertinent Lab Findings:
Defect in collagen synthesis (type III)
Buzz Words:
Cystic medial necrosis
Vascular rupture
Hyperelasticity
Ehlers-Danlos Syndrome
Clinical Presentation:
Elderly women (>50 years old).
Unilateral headache, jaw claudication, visual disturbances.
Flu-like symptoms with polymyalgia rheumatica.
Pertinent Lab Findings:
↑ ESR, ↑ CRP.
Segmental granulomatous inflammation of carotid branches.
Pathophysiology:
Th1 cell activation, IFN-γ, macrophages release MMPs.
Giant cells form in the arterial wall.
Buzz Words:
Granulomas, Giant cells, Risk of blindness.
Temporal artery biopsy needed.
Temporal (Giant Cell) Arteritis
Clinical Presentation:
Young Asian females (<50 years old).
Weak or absent upper extremity pulse (“Pulseless disease”).
Visual and neurological symptoms.
Pertinent Lab Findings:
↑ ESR, ↑ CRP.
Granulomatous inflammation of aortic arch and branches.
Pathophysiology:
Cell-mediated immunity (T cells, granuloma formation, MMP activation).
Buzz Words:
Pulseless disease, Granulomatous thickening, Aortic arch involvement.
Takayasu Arteritis
Clinical Presentation:
Young adults.
Hypertension (renal arteries), abdominal pain (mesenteric ischemia), skin ulcers.
Pertinent Lab Findings:
HBsAg associated (hepatitis B).
Transmural inflammation with fibrinoid necrosis.
Lesions of varying stages (“string-of-pearls” on imaging).
Pathophysiology:
Immune complex-mediated vasculitis.
Buzz Words:
Necrotizing vasculitis, Renal and visceral arteries, String-of-pearls.
Polyarteritis Nodosa (PAN)
Clinical Presentation:
Asian children < 4 years old
Fever > 5 days, conjunctivitis, rash (palms/soles), cervical lymphadenopathy
Coronary artery involvement → risk of MI
Pertinent Lab Findings:
↑ ESR
No ANCA association
Buzz Words:
Strawberry tongue
Coronary artery aneurysm
Aspirin + IVIG treatment
Kawasaki Disease
Clinical Presentation:
Heavy smokers
Ulceration, gangrene, autoamputation of fingers/toes
Raynaud phenomenon
Pertinent Lab Findings:
Segmental thrombosing vasculitis
Buzz Words:
Necrotizing vasculitis
Smokers
Autoamputation
Buerger Disease (Thromboangiitis Obliterans)
Clinical Presentation:
Middle-aged male.
Sinusitis, hemoptysis, hematuria (glomerulonephritis).
Upper respiratory + lung + kidney involvement.
Pertinent Lab Findings:
c-ANCA positive (PR3-ANCA).
Necrotizing granulomas.
Pathophysiology:
Neutrophil-mediated microabscess formation.
Buzz Words:
Saddle nose deformity, PR3-ANCA, Necrotizing granulomas.
Wegener Granulomatosis (Granulomatosis with Polyangiitis)
Clinical Presentation:
Men, 40–60 years old.
Pulmonary hemorrhage + glomerulonephritis.
Pertinent Lab Findings:
p-ANCA positive (MPO-ANCA).
Necrotizing vasculitis WITHOUT granulomas.
Pathophysiology:
Neutrophil degranulation triggered by ANCA.
Buzz Words:
Same-age lesions, p-ANCA, No granulomas.
Microscopic Polyangiitis
Clinical Presentation:
Asthma, allergic rhinitis.
Peripheral eosinophilia, sinusitis, neuropathy.
Pertinent Lab Findings:
p-ANCA positive (MPO-ANCA).
Eosinophilic infiltrates + granulomas.
Pathophysiology:
Th2 cell-driven eosinophilic inflammation.
Buzz Words:
Asthma + eosinophils + vasculitis, Cardiac involvement.
Churg-Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis)
Clinical Presentation:
Children (~6 years old), often post-URI.
Palpable purpura (buttocks/legs), GI pain, hematuria (IgA nephropathy).
Pertinent Lab Findings:
IgA immune complex deposition.
Pathophysiology:
Immune complex-mediated vasculitis.
Buzz Words:
IgA nephropathy, Self-limited, May recur.
Henoch-Schönlein Purpura
Clinical Presentation:
Hypersensitivity reaction (rash, fever)
Can mimic other vasculitides
Pertinent Lab Findings:
p-ANCA positive
Leukocytoclastic vasculitis
Buzz Words:
Penicillin, sulfa drugs
Cutaneous purpura
Drug-Induced Vasculitis
Clinical Presentation:
Adults 45–65 years old, Female predominance.
Associated with Hepatitis C.
Skin purpura, arthralgias, peripheral neuropathy.
Pertinent Lab Findings:
Cryoglobulin deposits.
Buzz Words:
Cold-precipitated immune complexes, Hyperviscosity syndrome.
Cryoglobulinemic Vasculitis
Clinical Presentation:
Recurrent oral and genital ulcers.
Eye involvement (uveitis → vision loss).
Neurological symptoms, arthritis, vasculitis.
Pertinent Lab Findings:
HLA-B51 association.
Pathophysiology:
Th1-driven inflammatory response, Neutrophilic vascular reaction.
Buzz Words:
HLA-B51, Oral/genital ulcers, Uveitis.
Behcet’s Disease
Clinical Presentation:
Dry eyes, dry mouth.
Parotid gland enlargement, increased lymphoma risk.
Pertinent Lab Findings:
Anti-Ro/SSA, Anti-La/SSB.
Buzz Words:
Lymphocytic sialadenitis, Rheumatoid association.
Sjogren’s Syndrome (Vasculitis Component)
A 72-year-old woman presents with unilateral headache and jaw pain while chewing. She complains of scalp tenderness and intermittent vision loss in her right eye. Physical exam reveals a thickened, tender temporal artery. Laboratory results show elevated ESR. A temporal artery biopsy is performed, showing granulomatous inflammation with multinucleated giant cells.
Temporal (Giant Cell) Arteritis (GCA)
A 28-year-old Asian woman presents with fatigue, weight loss, and night sweats. Over the past several months, she has experienced dizziness, arm pain with use, and visual disturbances. Blood pressure is markedly different between her arms, and pulses in her upper extremities are diminished. Aortic angiography reveals narrowing of major aortic branches.
Takayasu Arteritis
A 40-year-old man presents with uncontrolled hypertension, abdominal pain after meals, and bilateral foot drop. He reports recent episodes of skin rashes that appear as livedo reticularis (mottled, purplish skin discoloration). His medical history includes hepatitis B infection. Renal angiography shows multiple small aneurysms and areas of stenosis in medium-sized arteries.
Polyarteritis Nodosa (PAN)
A 3-year-old Asian boy is brought to the emergency room with five days of fever. He has bilateral conjunctival injection, erythematous cracked lips, and a strawberry tongue. His hands and feet appear red and swollen, and his skin is peeling. He has a diffuse, polymorphous rash and cervical lymphadenopathy. Laboratory findings show elevated ESR and CRP.
Kawasaki Disease
A 35-year-old male smoker presents with severe pain in his fingers and toes. He reports a history of cold sensitivity, where his fingers turn white, then blue, then red. He has developed painful ulcers on his fingertips, and physical examination shows gangrene of the right second toe. Angiography reveals occlusion of small and medium-sized arteries with corkscrew collaterals.
Buerger Disease (Thromboangiitis Obliterans)
A 50-year-old man presents with chronic sinus congestion, nosebleeds, and hearing loss. Over the past two months, he has developed shortness of breath and a cough with blood-streaked sputum. He also complains of dark urine. Urinalysis reveals red blood cell casts, and serum testing shows c-ANCA positivity. A lung biopsy demonstrates necrotizing granulomatous inflammation.
Granulomatosis with Polyangiitis (GPA, Wegener’s)
A 45-year-old man presents with progressive shortness of breath and hemoptysis. He also reports dark urine and new-onset hypertension. His renal function is worsening, and a urinalysis reveals red blood cell casts. Laboratory findings show p-ANCA positivity. A kidney biopsy demonstrates necrotizing vasculitis without granulomas.
Microscopic Polyangiitis (MPA)
A 38-year-old man with a history of asthma and allergic rhinitis presents with worsening shortness of breath and a skin rash. He reports numbness and weakness in his left foot. Laboratory results show eosinophilia and p-ANCA positivity. A nerve biopsy reveals necrotizing vasculitis with eosinophilic infiltrates.
Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg-Strauss)
A 6-year-old boy presents with palpable purpura on his buttocks and legs, abdominal pain, and recent joint pain. His parents state that he had a cold two weeks ago. Urinalysis shows microscopic hematuria and mild proteinuria. A skin biopsy reveals IgA immune complex deposition in small blood vessels.
Henoch-Schönlein Purpura (IgA Vasculitis)
A 55-year-old woman with a history of hepatitis C presents with purple, non-blanching skin lesions on her lower extremities and complaints of joint pain and peripheral neuropathy. Laboratory tests reveal cryoglobulinemia, low complement levels, and positive rheumatoid factor.
Cryoglobulinemic Vasculitis
A 30-year-old Middle Eastern man presents with recurrent painful oral ulcers and genital ulcers that resolve and recur. He also has intermittent blurry vision and eye pain. Physical exam reveals erythematous pustules at a site of previous skin trauma (positive pathergy test).
Behcet’s Disease
A 65-year-old woman started taking a new antibiotic (penicillin or sulfa drug) two weeks ago. She now presents with palpable purpura on her lower extremities, low-grade fever, and joint pain. Laboratory testing shows p-ANCA positivity, and a skin biopsy reveals leukocytoclastic vasculitis.
Drug-Induced Vasculitis
