Unit 2 Vasculitis Flashcards
Vasculititides
Define vasculitis and 3 etiological classifications
Inflammation of blood vessel wall. 1) Primary systemic vasculitis- specific cause unknown- presumed “immune mediated” (most of them). 2) Infectious (or direct) vasculitis—i.e. syphilis, RMSF, Gonorrhea, Mycoses (Aspergillus or Mucor). 3) Secondary systemic vasculitis—results from another disease process—i.e. HBV associated, Drug related HSR (ex: PCN, sulfa, etc…), Paraneoplastic, Lupus or RA immune complex mediated, Serum sickness
Describe the 3 layers of the BV wall
Intima-innermost layer; Media-smooth muscle layer in middle; Adventitia—outermost connective tissue layer.
Describe the nonspecific symptoms of vascular inflammation
Fever, wt loss, fatigue, myalgias
Describe the organ specific symptoms of vascular inflammation
Luminal narrowing or thrombosis can lead to organ ischemia
Large vessel vasculitides (LVV) include which vessels?
Aorta and major branches—Brachiocephalic, Subclavian, Common Carotid Arteries.
Medium vessel vasculitides (MVV) include which vessels?
Muscular arteries supplying organs—i.e. renal artery
Small vessel vasculitides (SVV) include which vessels?
Arterioles, capillaries and venules
List 2 major large vessel vasculitides (LVV)
Giant Cell Arteritis, Takayasu Arteritis
List 3 major medium vessel vasculitides (MVV)
Polyarteritis Nodosa, Kawasaki disease, Buerger disease
List 4 major small vessel vasculitides (SVV)
Wegener Granulomatosis, Microscopic Polyangiitis, Churg-Strauss Syndrome, Henoch-Schonlein Purpura
Describe the classic symptoms of Giant (Temporal) Cell Arteritis
Headache (HA)—temporal artery involved; Visual disturbance—opthalmic artery involved; Jaw claudication—maxillary artery; Flu-like symptoms, joint and muscle pain (polymyalgia rheumatica);
Most commonly affected arteries in Giant Cell Arteritis
External Carotid Artery branches: Superficial Temporal Artery, Maxillary Artery; Internal Carotid branches: Opthalmic Artery; Subclavian branch: Vertebral Artery
Most likely complication if GCA is NOT treated
Blindness
Treatment for GCA
Corticosteroids
GCA biopsy findings and interpretation
Inflamed vessel wall, giant cells, intimal fibrosis; long segments needed and negative biopsy does not exclude disease!
Describe the symptoms of Takayasu Arteritis (TAK)
Visual and neurological symptoms; weak or absent pulse in upper extremeity (pulseless)
Treatment for Takayasu arteritis (TAK)
Corticosteroids
List the major vessels commonly affected in TAK
Aortic arch at branch points
Name game: list another name for Wegeners Granulomatosis
Wegeners Granulomatosis = Granulomatosis with polyangiitis (GPA)
Name game: list another name for Churg-Strauss Syndrome
Churg-Strauss Syndrome = Eosinophilic granulomatosis with polyangiitis (EGPA)
Name game: list another name for Henoch-Schonlein Purpura
Henoch-Schonlein Purpura = IgA Vasculitis
Describe the classic symptoms of Polyarteritis Nodosa (PAN)
Young adults with hypertension (renal artery), abdominal pain with melena (mesenteric artery), neurologic disturbances and skin lesions.
Most likely complication if PAN is NOT treated
PAN is fatal if not treated
Treatment for PAN
Corticosteroids and Cyclophosphamide
Most commonly affected arteries in PAN
Medium sized, muscular arteries to all organ systems EXCEPT lungs.
Describe PAN blood vessel pathological findings
Necrotizing lesions—demonstrate fibrinoid necrosis where medial muscle and adjacent tissues are fused into a structureless eosinophilic mass that stains for fibrin. Inflammatory response/cells cause infarcts that produce symptoms.
Viral infections associated with PAN-like lesions
Hepatitis B Virus (associated with HBsAg), HCV, HIV
Imaging: PAN findings
“String of Pearls” appearances due to fibrotic nodes resulting from healed areas of necrosis in BV
Compare/contrast age, gender for GCA vs TAK
GCA patients > 50 yo and female; most common vasculitis. TAK patients < 50 yo and female (Asian)
Vasculitis commonly affecting children
Kawasaki disease (usually Asian child <4)
Describe classic symptoms of Kawasaki disease (KD)
Non-specific: fever, conjunctivitis, erythematous rash of palms and soles, oral lesions, enlarged cervical LN in a child
Most commonly affected arteries in Kawasaki disease (KD)
Coronary arteries
Major complications of Kawasaki disease (KD)
1)Thrombosis with MI or 2) Aneurysm with rupture
Treatment for Kawasaki disease (KD)
Usually self-limited; May treat with IVIG and aspirin
Name game: list another name for Kawasaki disease (KD)
Kawasaki disease = Mucocutaneous Lymph Node Syndrome
Describe the classic symptoms of Buerger disease
Ulceration, gangrene, auto-amputation of fingers and toes; Raynauds phenomenon is often present.
Major risk factor for Buerger disease
Smoking (heavy)
Treatment for Buerger disease
Smoking cessation
Name game: list another name for Buerger disease
Buerger disease = Thromboangiitis Obliterans
Describe the pathological mechanism of Buerger disease
Necrotizing vasculitis of digits: Smoking elicits pro-inflammatory antibodies; PMNs infiltrate arteries, neighboring veins while endothelium becomes inflamed leading to thrombosis and obliteration of BV lumen. Lesions become severe and progress to gangrene.
Describe classic symptoms of Wegener Granulomatosis
Middle aged male; Sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates (pneumonitis) and hematuria, proteinuria due to rapidly progressive glomerulonephritis (RPGN).
Most commonly affected arteries/organs in Wegeners Granulomatosis
Small arteries, veins or lung (respiratory tract), kidney and spleen.
Most likely complication if Wegeners Granulomatosis is NOT treated
Wegeners Granulomatosis is FATAL if untreated. Most will die within 1 year (avg: 5-6 months)
Treatment for Wegeners Granulomatosis
Cyclophosphamide and steroids; Relapses occur
Biopsy: Wegeners Granulomatosis
Large necrotizing granulomas with adjacent necrotizing vasculitis
Lab marker: Wegener Granulomatosis
Serum c-ANCA (aka anti-proteinase 3 or PR3-ANCA) correlates with disease activity; ANCA = antineutrophil cytoplasmic antigens
Describe classic symptoms of Microscopic Polyangiitis
Sinusitis, hemoptysis with nodular infiltrates, hematuria, proteinuria (presentation similar to Wegeners). No granulomas or nasopharyngeal involvement.
Lab marker: Microscopic Polyangiitis
Serum p-ANCA (aka anti-myeloperoxidase or MPO-ANCA) correlates with disease activity
Treatment for Microscopic Polyangiitis
Cyclophosphamide and corticosteroids. Relapse common
Describe classic patient type with Churg-Strauss Syndrome
Asthma patients; CSS is a systemic vasculitis of young patients with asthma.
Most commonly affected arteries/organs in Churg-Strauss Syndrome
Small vessels of lungs and heart; liver, kidney, spleen, CNS and other organs
Describe pathology of Churg-Strauss Syndrome
Necrotizing lesions of small vessels (granulomas) with intense eosinophilic infiltrates;
Treatment for Churg-Strauss Syndrome
Corticosteroids; poor prognosis if untreated
Lab marker: Churg-Strauss Syndrome
Serum p-ANCA (aka anti-myeloperoxidase or MPO-ANCA) correlates with disease activity
Describe classic symptoms of Henoch-Schonlein Purpura (HSP)
Child with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy) following a upper respiratory tract infection. Most common vasculitis in children
Describe pathology of HSP
Respiratory infection leads to IgA immune complex deposition in vessels—> vasculitis.
Treatment for HSP
Self-limited; Steroids for severe cases; may recur
