Etiology
Classification of Vasculitis (Based on Clinicopathologic Features): 1994 Chapel Hill Classification
Primary Idiopathic Vasculitis
- Small Vessel Vasculitis
- Churg-Strauss Syndrome (see Churg-Strauss Syndrome): systemic necrotizing vasculitis
- Idiopathic Capillaritis
- Microscopic Polyangiitis (see Microscopic Polyangiitis): systemic necrotizing vasculitis
- Pauci-Immune Glomerulonephritis (see Pauci-Immune Glomerulonephritis)
- Wegener’s Granulomatosis (see Wegeners Granulomatosis)
- Medium Vessel Vasculitis
- Buerger’s Disease (see Buerger’s Disease)
- Kawasaki Disease (see Kawasaki Disease)
- Polyarteritis Nodosa (see Polyarteritis Nodosa)
- Large Vessel Vasculitis
- Cogan’s Syndrome (see Cogan’s Syndrome)
- Takayasu’s Arteritis (see Takayasu’s Arteritis)
- Temporal Arteritis (see Temporal Arteritis)
Primary Small-Vessel, Immune Complex-Mediated Vasculitis
- Behcet’s Disease (see Behcet’s Disease)
- Goodpasture’s Syndrome (see Goodpasture’s Syndrome)
- Henoch-Schonlein Purpura (see Henoch-Schonlein Purpura)
- IgA Nephropathy (see IgA Nephropathy)
- Mixed Cryoglobulinemia (see Cryoglobulinemia)
Secondary Vasculitis
- Autoimmune + Connective Tissue Disease-Associated Vasculitis
- Anti-Phospholipid Antibody Syndrome (see Anti-Phospholipid Antibody Syndrome)
- Relapsing Polychondritis (see Relapsing Polychondritis)
- Rheumatoid Arthritis (see Rheumatoid Arthritis)
- Sjogren’s Syndrome (see Sjogren’s Syndrome)
- Systemic Lupus Erythematosus (SLE) (see Systemic Lupus Erythematosus)
- Drug-Induced Vasculitis
- All-Trans Retinoic Acid (see All-Trans Retinoic Acid)
- Mitomycin (see Mitomycin)
- Phenytoin (see Phenytoin)
- Propylthiouracil (see Propylthiouracil)
- Infection-Associated Vasculitis
- Chronic Active Hepatitis B (see Hepatitis B Virus)
- Chronic Epstein-Barr Virus (see Epstein-Barr Virus)
- Endocarditis (see Endocarditis)
- Malignancy-Associated (Paraneoplastic) Vasculitis
- Acute Myeloid Leukemia (AML) (see Acute Myeloid Leukemia)
- Adenocarcinoma
- Chronic Lymphocytic Leukemia (CLL) (see Chronic Lymphocytic Leukemia)
- Hairy Cell Leukemia (see Hairy Cell Leukemia)
- Hepatocellular Carcinoma (see Hepatocellular Carcinoma)
- Hodgkin’s Disease (see Hodgkin’s Disease)
- Lymphoma (see Lymphoma)
- Multiple Myeloma (see Multiple Myeloma)
- Myelodysplastic Syndrome (see Myelodysplastic Syndrome)
- Myeloproliferative Disorders (see Myeloproliferative Disorders)
- Chronic Eosinophilic Leukemia/Hypereosinophilic Syndrome (see Chronic Eosinophilic Leukemia and Hypereosinophilic Syndrome)
- Chronic Myeloid Leukemia (see Chronic Myeloid Leukemia)
- Chronic Neutrophilic Leukemia (see Chronic Neutrophilic Leukemia)
- Essential Thrombocytosis (see Essential Thrombocytosis)
- Polycythemia Vera (see Polycythemia Vera)
- Primary Myelofibrosis (see Primary Myelofibrosis)
- Systemic Mastocytosis (see Systemic Mastocytosis)
- Renal Cell Carcinoma (see Renal Cell Carcinoma)
- Other
- Inflammatory Bowel Disease (see Inflammatory Bowel Disease)
- Hypocomplementemic Urticarial Vasculitis (see Hypocomplementemic Urticarial Vasculitis)
Classification of Vasculitis (Based on Site of Vascular Involvement)
(Note: there can be some overlap between the site of vascular involvement in these disorders -> while small and medium-vessel vasculitides may also involve the medium-sized arteries, large-vessel vasculitides do not usually involve vessels smaller than arteries)
Large Vessel Vasculitis (involving aorta and arteries)
- Cogan’s Syndrome (see Cogan’s Syndrome)
- Takayasu’s Arteritis (see Takayasu’s Arteritis)
- Temporal Arteritis (see Temporal Arteritis)
Medium-Vessel Vasculitis (involving arteries and arterioles)
- Buerger’s Disease (see Buerger’s Disease)
- Kawasaki Disease (see Kawasaki Disease)
- Polyarteritis Nodosa (see Polyarteritis Nodosa)
Small-Vessel Vasculitis (involving arterioles, capillaries, venules, and veins)
- Connective Tissue Disease
- Mixed Connective Tissue Disease (MCTD) (see Mixed Connective Tissue Disease)
- Rheumatoid Arthritis (see Rheumatoid Arthritis)
- Sjogren’s Syndrome (see Sjogren’s Syndrome)
- Systemic Lupus Erythematosus (SLE) (see Systemic Lupus Erythematosus)
- Drug-Induced Vasculitis
- All-Trans Retinoic Acid (see All-Trans Retinoic Acid)
- Mitomycin (see Mitomycin)
- Phenytoin (see Phenytoin)
- Propylthiouracil (see Propylthiouracil)
- Hypersensitivity Vasculitis (Leukocytoclastic, Presumably Immune Complex-Mediated Vasculitis)
- Connective Tissue Disease-Associated Hypersensitivity Vasculitis
- Mixed Connective Tissue Disease (see Mixed Connective Tissue Disease)
- Relapsing Polychondritis (see Relapsing Polychondritis)
- Rheumatoid Arthritis (see Rheumatoid Arthritis)
- Sjogren’s Syndrome (see Sjogren’s Syndrome)
- Systemic Lupus Erythematosus (SLE) (see Systemic Lupus Erythematosus)
- Infection-Associated Hypersensitivity Vasculitis
- Chronic Active Hepatitis B (see Hepatitis B Virus)
- Chronic Epstein-Barr Virus (see Epstein-Barr Virus)
- Endocarditis (see Endocarditis)
- Malignancy-Associated Hypersensitivity Vasculitis
- Acute Myeloid Leukemia (AML) (see Acute Myeloid Leukemia)
- Chronic Lymphocytic Leukemia (CLL) (see Chronic Lymphocytic Leukemia)
- Hairy Cell Leukemia (see Hairy Cell Leukemia)
- Hepatocellular Carcinoma (see Hepatocellular Carcinoma)
- Hodgkin’s Disease (see Hodgkin’s Disease)
- Lymphoma (see Lymphoma)
- Multiple Myeloma (see Multiple Myeloma)
- Myelodysplastic Syndrome (see Myelodysplastic Syndrome)
- Myeloproliferative Disorders (see Myeloproliferative Disorders)
- Chronic Eosinophilic Leukemia/Hypereosinophilic Syndrome (see Chronic Eosinophilic Leukemia and Hypereosinophilic Syndrome)
- Chronic Myleoid Leukemia (see Chronic Myeloid Leukemia)
- Chronic Neutrophilic Leukemia (see Chronic Neutrophilic Leukemia)
- Essential Thrombocytosis (see Essential Thrombocytosis)
- Polycythemia Vera (see Polycythemia Vera)
- Primary Myelofibrosis (see Primary Myelofibrosis)
- Systemic Mastocytosis (see Systemic Mastocytosis)
- Renal Cell Carcinoma (see Renal Cell Carcinoma)
- Other
- Congenital Complement Deficiency
- Alpha-1 Antitrypsin Deficiency (see Alpha-1 Antitrypsin Deficiency)
- Henoch-Schonlein Purpura (see Henoch-Schonlein Purpura>)
- Mixed Cryoglobulinemia (see Cryoglobulinemia)
- Post-Intestinal Bypass
- Primary Biliary Cirrhosis (see Primary Biliary Cirrhosis)
- Retroperitoneal Fibrosis
- Serum Sickness/Serum Sickness-Like Reaction (see Serum Sickness)
- Ulcerative Colitis (see Ulcerative Colitis)
- Connective Tissue Disease-Associated Hypersensitivity Vasculitis
- Other
- Anti-Phospholipid Antibody Syndrome (see Anti-Phospholipid Antibody Syndrome)
- Behcet’s Disease (see Behcet’s Disease)
- Churg-Strauss Syndrome (see Churg-Strauss Syndrome): systemic necrotizing vasculitis
- Goodpasture’s Syndrome (see Goodpasture’s Syndrome)
- Hypocomplementemic Urticarial Vasculitis (see Hypocomplementemic Urticarial Vasculitis)
- IgA Nephropathy (see IgA Nephropathy)
- Microscopic Polyangiitis (see Microscopic Polyangiitis): ANCA-associated systemic necrotizing vasculitis
- Pauci-Immune Glomerulonephritis (see Pauci-Immune Glomerulonephritis)
- Wegener’s Granulomatosis (see Wegener’s Granulomatosis): ANCA-associated vasculitis
Clinical Features
Features of Large Vessel Vasculitis
- Limb Claudication
- Asymmetric BP’s
- Absence of Pulses
- Bruits
- Aortic Dilation
Features of Medium-Vessel Vasculitis
- Cutaneous Nodules (see Papular-Nodular Skin Lesions)
- Ulcers
- Livedo Reticularis (see Livedo Reticularis)
- Digital Gangrene
- Mononeuritis Multiplex (see Peripheral Neuropathy)
- Microaneurysms
- Episcleritis/Scleritis
Features of Small-Vessel Vasculitis
- Purpura (see Purpura)
- Vesiculobullous Lesions (see Vesicular-Bullous Skin Lesions)
- Urticaria (see Urticaria-Angioedema)
- Glomerulonephritis (see Acute Glomerulonephritis)
- Diffuse Alveolar Hemorrhage (see Diffuse Alveolar Hemorrhage)
- Cutaneous Extravascular Necrotizing Granulomas
- Splinter Hemorrhages (see Splinter Hemorrhages)
- Uveitis
Differentiating Features of Vasculitis Syndromes
Wegener’s Granulomatosis (see Wegeners Granulomatosis)
- Pathology
- Site: small-medium arterioles to venules (sometimes arteries and veins) -> necrotizing
- Capillaritis
- Immunofluorescence: granular or negative
- Serology
- c-ANCA: positive (>1:20) in 80-90% of cases (high titers correlate well with active granulomatous vasculitis)
- c-ANCA (anti-proteinase 3 = anti-PR3) ELISA positive in >85% of cases with generalized active disease
- ANA: variable
- RF: variable
- Complement: normal
- c-ANCA: positive (>1:20) in 80-90% of cases (high titers correlate well with active granulomatous vasculitis)
- Clinical
- Constitutional: fatigue, weight loss, malaise, fevers
- Upper Respiratory Tract (70-95% of cases): destructive or ulcerating lesions -> nasal septal perforation, saddle-nose deformity, subglottic stenosis
- Lung (70-95% of cases): lung nodules, cavitary lesions, DAH, trachebronchial disease (10-50% of cases)
- Chest imaging is abnormal in 80% of cases: alveolar, interstitial, mixed alveolar-interstitial, nodules, cavitary disease, tracheobronchial lesions
- Renal (50-90% of cases): necrotizing crescentic GLN, occasional granulomatous features, absence of HTN
- GI (<10% of cases):
- Rheum (80% of cases): arthritis, arthralgias, myalgias, synovitis
- Derm (60% of cases): skin vasculitis with palpable purpura, ulcers, nodules, vesicles
- Neuro (may be involved): mononeuritis multiplex, CNS involvement
- Ocular (20-60% of cases): uveitis, ocular ulcers
- Cardiac (5-30% of cases):
- Vascular: microaneurysms are rare
- Distinguishing Features
- Destructive upper airway disease
- Granulomatous inflammation
Microscopic Polyangiitis (see Microscopic Polyangiitis)
- Epidemiology
- Most common cause of pulm-renal syndromes (several times more common than Goodpasture’s)
- Pathology
- Site: small-medium arterioles to venules (sometimes arteries and veins) -> necrotizing
- Capillaritis
- Immunofluorescence: negative
- Serology
- p-ANCA (anti-myeloperoxidase = anti-MPO) : ANCA positive in 50-75% of cases
- ANA: variable
- RF: variable
- Complement: normal
- Clinical
- Constitutional: very common (generally precedes renal disease by months)
- Upper Respiratory Tract (5-30% of cases): usually mild involvement -> sinus disease
- Lung (25-55% of cases)
- DAH (10-30% of cases)
- Pulmonary fibrosis
- Focal infiltrates (10-30% of cases)
- Pleural effusion (5-20% of cases)
- Renal: necrotizing crescentic GLN, absence of HTN -> rapidly progresive glomerulonephritis (almost universal)
- GI (35-55% of cases): abdominal pain, GI bleeding, ischemia/infarction, visceral aneurysms (rare)
- Rheum (50% of cases): arthritis, arthralgias, myalgias
- Derm (35-60% of cases): skin vasculitis with purpura
- Neuro: mononeuritis multiplex (common): mononeuritis multiplex in 10-50% of cases
- Ocular (0-30% of cases): may be clinically silent
- Cardiac (10-15% of cases): CHF, pericarditis
- Vascular: microaneurysms are rare
- Distinguishing Features
- Absence of granulomatous inflammation
Churg-Strauss Syndrome (see Churg-Strauss Syndrome)
- Pathology
- Site: systemic necrotizing vasculitis
- Serology
- p-ANCA (anti-myeloperoxidase = anti-MPO) : ANCA positive in 40-70% of cases
- Clinical
- Constitutional: common
- Upper Respiratory Tract (20-70% of cases): usually non-destructive -> sinusitis, nasal polyps, allergic rhinitis
- Lung: asthma (universally present), obstructive airways disease (airway wall thickening, hyperinflation), eosinophilic fleeting infiltrates (30-50% of cases), DAH (0-20% of cases)
- Chest imaging: opacities in 40-75% of cases
- Renal (20-60% of cases): necrotizing crescentic GLN (severe renal disease is unusual)
- GI (15-60% of cases, major cause of morbidity and mortality): GI bleed, abdomnal pain, ischemia/infarction, perforation
- Rheum (50% of cases): arthritis, arthralgias, myalgias
- Derm (50-70% of cases): nodules, papules, leukocytoclastic vasculitis with or without eosinophils
- Neuro: mononeuritis multiplex in 50-75% of cases, CNS involvement in 5-40% of cases
- Ocular (<5% of cases):
- Cardiac (15-50% of cases): conduction delays, EKG abnormalities, systolic or diatolic CHF, pericarditis, coronary artery vasculitis
- Distinguishing Features
- Allergy
- Eosinophilia
Polyarteritis Nodosa (see Polyarteritis Nodosa)
- Pathology
- Site: medium muscular arteries -> necrotizing
- Serology
- ANCA: negative
- Clinical
- Lung: absence of involvement
- Renal: absence of glomerulonephritis, presence of HTN
- GI: presence of GI symptoms
- Derm: involved
- Neuro: mononeuritis multiplex (common)
- Ocular: absence of symptoms
- Vascular: microaneurysms are common
- Distinguishing Features
- Absence of granulomatous inflammation
Systemic Lupus Erythematosus (SLE) (see Systemic Lupus Erythematosus)
- Pathology
- Bland or capillaritis
- Immunofluorescence: granular IgG
- Serology
- ANCA: negative
- ANA: positive (>1:160 titer) in 95% of cases
- Anti-DNA: positive
- RF: positive
- Complement: decreased
- Clinical
- Lung: DAH
- Renal: involved
- Rheum: arthritis
- Derm: skin vasculitis (variable)
Goodpasture’s Syndrome (see Goodpasture’s Syndrome)
- Pathology
- Bland or capillaritis
- Immunofluorescence: linear IgG
- Serology
- ANCA: negative
- ANA: negative
- RF: negative
- Complement: normal
- Anti-Basement Membrane: positive
- Clinical
- Lung: DAH
- Renal: involved
Idiopathic Pulmonary Hemosiderosis (see Idiopathic Pulmonary Hemosiderosis)
- Pathology
- Bland
- Immunofluorescence: negative
- Serology
- ANCA: negative
- ANA: negative
- RF: negative
- Complement: normal
- Clinical
- Lung: DAH
- Heme: anemia
Isolated Pulmonary Capillaritis (see Isolated Pulmonary Capillaritis)
- Pathology
- Capillaritis
- Immunofluorescence: negative
- Serology
- ANCA: usually negative (occasionally p-ANCA positive)
- ANA: negative
- RF: negative
- Complement: normal
- Clinical
- Lung: DAH
- Heme: anemia
Henoch-Schonlein Purpura (see Henoch-Schonlein Purpura)
- Pathology
- Immunofluorescence: granular IgA
- Serology
- ANCA: variable
- ANA: negative
- RF: negative
- Complement: normal
- IgA Immune Complexes: positive
- Clinical
- Lung: DAH
- Renal: involved
- Rheum: arthritis
- Derm: skin vasculitis
Type II or III Mixed Cryoglobulinemia (see Cryoglobulinemia)
- Clinical
- Renal: glomerulonephritis
- Neuro: peripheral neuropathy
- Derm: purpura
- Distinguishing Features
- Most type II and III cases are associated with HCV infection -> antiviral therapy can cause remission in some patients
Rheumatoid Vasculitis (see Rheumatoid Arthritis)
- Clinical
- Cardiac: pericarditis
- Lung: pleuritis
- Renal:
- Derm: skin vasculitis
- Vascular: digital ischemia
- Neuro: peripheral neuropathy
- Ocular: scleritis
References
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