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39 Cards in this Set
- Front
- Back
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29 yo woman presents to primary care physician with no complaints but found to have no blood pressure in left arm
• 75 yo man complains of headaches, upper extremity pain/weakness, right eye blindness • 4 yo child with rash and myocardial infarction • 43 yo woman admitted from ER with hemoptysis, shortness of breath, bloody urine • 45 yo man with rash on legs and history of liver disease Differential? |
VASCULITIS
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MANIFESTATIONS OF
SYSTEMIC VASCULITIS What are some specific signs/symptoms related to vessels involved? (2) |
• Non-specific signs and symptoms
reflecting systemic inflammatory disease • Specific signs and symptoms related to vessels involved – Ischemia (insufficient blood supply) – Infarction (tissue death) |
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CLASSIFICATION OF VASCULITIS
Which is most useful in relating clinical signs and symptoms to specific disorder? |
• Anatomical size of vessel involved
– Most useful in relating clinical signs and symptoms to specific disorder |
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SIZE CLASSIFICATION OF
SYSTEMIC VASCULITIS Large vessel vasculitis? GATA Medium vessel vasculitis? PK Small vessel vasculitis? HEWCM |
LARGE-VESSEL VASCULITIS
Giant-cell (temporal) arteritis Takayasuʼs arteritis MEDIUM-SIZED-VESSEL VASCULITIS Polyarteritis nodosa Kawasakiʼs disease SMALL VESSEL VASCULITIS Henoch-Scholein purpura Essential cryoglobulinemic vasculitis Wegenerʼs granulomatosis Churg-Strauss syndrome Microscopic polyangiitis |
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CHARACTERISTICS OF SYSTEMIC
LARGE VESSEL VASCULITIC SYNDROMES Vasculitis: name of disease for large artery? Granulomatous arteritis of ______ and ______ branches, especially ____________ branches of carotid Usually patient > ____ years old Associated with? Usually involves _____ artery May lead to ________ (eye) |
aorta
major extra cranial 50 Associated with PMR, headache, jaw, or tongue claudication Temporal Blindness |
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TEMPORAL ARTERITIS AND PMR
Polymyalgia rheumatica (PMR) – Signs/symptoms? – Disease of what age group? – ESR level? • What biopsy should you consider with these patients? What is treatment of temporal arteritis? |
Non-specific “weakness” of shoulder girdle without
evidence of muscle inflammation (normal CPK); pain limits muscle use Disease of elderly Elevated erythrocyte sedimentation rate (ESR) Consider temporal artery biopsy • Treatment dependent on presence of temporal arteritis (high dose corticosteroids) – Without temporal arteritis, may be treated with NSAIDs or low doses of corticosteroids (prompt relief) |
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VASCULITIS
Definition? |
Group of heterogeneous disorders involving inflammation of blood vessel walls
– Important to note that these disorders are syndromes • Association of signs, symptoms, and pathologic findings • Few pathognomonic findings – Nevertheless, disorders can be distinguished |
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GIANT CELL ARTERITIS FINDINGS:
3? |
Retinal Ischemia
Scalp Necrosis Dilated temporal branches (may be indurated) |
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CHARACTERISTICS OF SYSTEMIC
LARGE VESSEL VASCULITIC SYNDROMES VASCULITIS: Takayasu's Arteritis DEFINITION: - which branches? COMMENTS Common age? Ethnic/location? Frequent presentation? Predominant area of aorta affected? |
Granulomatous inflammation of aorta and major branches
Usually patient <50 More common in Japan Frequently presents with "pulse-less" disease or vascular insufficiencies of upper extremities May affect ANY area of aorta |
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Key Points: Large Vessel
Vasculitis Compare Giant Cell (Temporal) Arteritis with Takayasu's arteritis age? associated with systemic complaints? which artery(s) commonly affected? Major problem? |
Giant cell (temporal) arteritis
– Elderly – Associated with PMR (polymyalgia rheumatica) – Temporal artery most often affected – Major problem-blindness Takayasuʼs arteritis – Young women, especially Asian – Often without systemic complaints – Aorta and large branches – Major problem-large vessel insufficiency |
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CHARACTERISTICS OF
MEDIUM-SIZE VESSEL VASCULITIC SYNDROMES Vasculitis: Polyarteritis Nodosa DEFINITION: Does it involve glomerulonephritis or vasculitis in arterioles, caps, venules? Comments: Associated with which Hepatitis? Skin? Neuropathy? Which arteries? |
No, does not involve glomerulonephritis or vasculitis in arterioles, caps, venules
Hepatitis B Skin Nodules Mononeuritis Multiplex (wrist or foot drop) Frequently involves mesenteric arteries |
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Polyarteritis nodosa:
Common presentations? (3) |
hand, subcutaneous nodules
wrist drop (this neuropathy is due to ischemia of the vasa nervorum) multiple saccular aneurysms in superior mesenteric artery |
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CHARACTERISTICS OF
MEDIUM-SIZE VESSEL VASCULITIC SYNDROMES Vasculitis: Kawasaki's disease Definition: Arteritis involving? Comments: Age group affected? Associated with what syndrome? Arteries involved? |
Arteritis involving large, medium, and small arteries (Aorta and veins may be involved)
Usually occurs in children Associated with mucocutaneous lymph node syndrome Coronary arteries often involved |
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Kawasaki disease
Common Manifestations? |
polymorphous macular exanthem is one of the manifestations of
Kawasaki disease (mucocutaneous lymph node syndrome) Diffuse macular areas Fissured, cracked, and painful cherry red lips Redness of palms and soles with subsequent peeling strawberry tongue skin desquamation coronary artery aneurysm coronary artery thrombus |
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Key Points: Medium Vessel Vasculitis
Polyarteritis nodosa – Hepatitis? – Wrist/foot? – Which arteritis Kawasakiʼs Disease – age group? – syndrome? – which arteritis |
Polyarteritis nodosa
– Hepatitis B – Wrist/foot drop (mononeuritis multiplex) – Mesenteric arteritis Kawasakiʼs Disease – Children – Mucocutaneous LN syndrome – Coronary arteritis |
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ORGAN INVOLVEMENT IN SMALL VESSEL VASCULITIS
Name 6: |
Skin
Kidney Lung Neurologic GI Musculoskeletal |
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ORGAN INVOLVEMENT IN SMALL VESSEL VASCULITIS
Skin: |
Leukocytoclastic vasculitis (palpable purpura)
– Leukocyte infiltrate of vessel wall with leukocytoclasis (nuclear degeneration) |
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ORGAN INVOLVEMENT IN SMALL VESSEL VASCULITIS
Kidney: Protein? Casts? Cr? |
Glomerulonephritis (proteinuria, RBC casts, elevated creatinine)
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ORGAN INVOLVEMENT IN SMALL VESSEL VASCULITIS
Lung: Infiltrates present? Hemorrhage common? Other findings? |
Pulmonary infiltrates or hemorrhage (abnormal chest x-ray)
– Upper airway (ENT): Mucosal inflammation; sinusitis (nasal discharge, saddle nose, abnormal sinus films) |
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ORGAN INVOLVEMENT IN SMALL VESSEL VASCULITIS
Neurologic: what is affected? Gastrointestinal: |
Neuro: Inflammation of vasonervorum leading to nerve infarction and polyneuropathy
GI: Non-specific nausea, vomiting, diarrhea |
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ORGAN INVOLVEMENT IN SMALL VESSEL VASCULITIS
Musculo-skeletal: common findings? |
Non-specific myalgias, arthralgias,
arthritis |
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What Lab test do we order for small cell vasculitis?
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ANCA:
- pANCA - cANCA |
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TWO ANCA PATTERNS
• Cytoplasmic ANCA (cANCA) - directed against? - most often associated with which small cell vasculitis? • Perinuclear ANCA (pANCA) - directed against? - most often associated with which small cell vasculitis? |
• Cytoplasmic ANCA (cANCA)
– Directed against proteinase 3 – Frequently associated with Wegenerʼs granulomatosus • Perinuclear ANCA (pANCA) – Directed against myeloperoxidase – Most often associated with microscopic polyangiitis or Churg- Strauss syndrome |
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Asthma and eosinophilia are associated with which ONE of the small cell vasculitis?
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Churg-Strauss
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CLINICAL FEATURES
SUGGESTING VASCULITIS - focal/diffuse/mutli-system? - course/progression? - constitutional symptoms? - ESR? anemia? - Antibody production? - Skin? - Lung? - Neuro? - Kidney? |
• Multi-system inflammatory disorder
• Rapidly progressive major organ dysfunction • Constitutional symptoms (fever, weight loss) • High ESR, severe anemia, thrombocytosis • Autoantibody production • Evidence of small vessel inflammation – Kidney: Proteinuria, RBC casts, elevated creatinine – Lungs: Hemoptysis, dyspnea – Skin: Palpable purpura, hemorrhage • Acute neurologic changes – Footdrop – Altered mental status |
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DIFFERENTIAL DIAGNOSIS OF VASCULITIS
Infections? Embolic disorders? Malignancy? Drug-induced? |
• Infections
– Viral (HIV, hepatitis), bacterial, Lyme disease – Infective endocarditis • Embolic disorders – Infective endocarditis – Left atrial myxoma – Cholesterol emboli • Malignancy – Hairy cell leukemia • Drug-induced – Ergots – Cocaine – Amphetamines |
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DIAGNOSTIC EVALUATION OF
VASCULITIS (AND MIMICS) • Routine laboratory evaluation – CBC (2) – ESR and/or CRP (elevated/low?) – Blood cultures (positive/negative?) – Liver function tests (mild ________ common except when associated with hepatitis) – Hepatitis antigen/antibodies (______ except Hep_ or _) – Renal function (______ Cr, BUN) – Urinalysis (3) – Urine toxicology screen (pos/neg?) • Serologic evaulation (diagnostic?) – ANCA (positive/negative?) – ANA (____ titer positive) – ENA (Sm, RNP) (pos/neg?) – Anti-dsDNA (pos/neg) |
DIAGNOSTIC EVALUATION OF
VASCULITIS (AND MIMICS) • Routine laboratory evaluation – CBC (anemia, thrombocytosis) – ESR and/or CRP (elevated) – Blood cultures (negative) – Liver function tests (mild elevations common except when associated with hepatitis) – Hepatitis antigen/antibodies (negative except HepB or C) – Renal function (increased Cr, BUN) – Urinalysis (hematuria, casts, proteinuria) – Urine toxicology screen (negative) • Serologic evaulation (usually not diagnostic) – ANCA (maybe positive) – ANA (low titer positive) – ENA (Sm, RNP) (negative) – Anti-dsDNA (negative) |
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DIAGNOSTIC EVALUATION OF
VASCULITIS • Radiologic imaging studies – Chest (?) – Sinus (2) • Echocardiogram – Rule out ______? (2) and atrial _____? |
• Radiologic imaging studies
– Chest (Pulmonary infiltrates) – Sinus (Mucosal thickening, sinusitis) • Echocardiogram – Rule out vegetations (Libman-Sacks endocarditis of SLE, infective endocarditis) and atrial myxoma |
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DIAGNOSIS OF VASCULITIS
Usually requires ______ of involved tissue revealing pathologic findings of vasculitis Characteristic mesenteric artery angiographic findings may be helpful for what condition? |
biopsy
Polyarteritis nodosa (saccular aneurysms, beaded appearance, skip lesions) |
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GENERAL TREATMENT STRATEGY: IMMUNSUPPRESSION
• Corticosteroids – Daily (?) – Pulse doses (?) • Cytotoxic drugs – __________ (IV or po), especially for Wegenerʼs and life-threatening vasculitis – Methotrexate – Azathioprine |
• Corticosteroids
– Daily (IV or po) – Pulse doses (IV) • Cytotoxic drugs – Cyclophosphamide (IV or po), especially for Wegenerʼs and life-threatening vasculitis – Methotrexate – Azathioprine |
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COMPLICATIONS OF THERAPY
• Drug toxicities: Cyclophosphamide in Wegenerʼs: |
• Drug toxicities: Cyclophosphamide in Wegenerʼs
- Hemorrhagic cystitis and bladder carcinoma - Bone marrow suppression and myelodysplastic syndromes - Malignancy |
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CLASSIFICATION OF
HERPESVIRUSES • ALPHA HERPESVIRUSES? CLASSIFICATION OF HERPESVIRUSES • ALPHA HERPESVIRUSES? • BETA HERPESVIRUSES? • GAMMA HERPESVIRUSES? |
• ALPHA HERPESVIRUSES
– Herpes simplex, type I and II – Varicella-zoster • BETA HERPESVIRUSES – Cytomegalovirus – Human herpesvirus 6 (HHV6) – HHV7 • GAMMA HERPESVIRUSES – Epstein-Barr virus – HHV8 (Kaposiʼs sarcoma-associated herpesvirus, KSHV) – γHV68 |
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CRYOGLOBULINEMIA
Three classic types – Type I: what kind of antibody? – Type II:what kind of antibody? – Type III: what kind of antibody? |
• Presence of serum cryoglobulins
– Precipitates formed when serum is refrigerated. • Three classic types – Type I: Monoclonal immunoglobulin – Type II: Monoclonal immunoglobulin with activity against polyclonal IgG – Type III: Mixed polyclonal IgG |
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CLINICAL MANIFESTATIONS
OF CRYOGLOBULINEMIA Skin? (2) Kidneys? (1) Small vessels? (2) |
• Immune complex deposition in skin
– “Palpable purpura” or leukocytoclastic vasculitis • Immune complex deposition in kidneys – Glomerulonephritis • Vascular occlusive phenomena (small vessels) – Raynaudʼs phenomenon – Distal infarcts |
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PREVIOUS TREATMENT OF
CRYOGLOBULINEMIA (3) |
• Immunosuppressive drugs
• Plasmapheresis • Penicillamine? |
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HEPATITIS C VIRUS
RNA/DNA virus? Course? High/low grade damage? Carrier state - chronic or acute? Antibody production? |
• RNA virus related to flaviviruses,
pestiviruses • Hepatitis characterized by chronic, indolent course – Low grade hepatic damage – Chronic carrier state; >20 years – Autoantibody production |
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Cryoglobulinemia is mediated by what type of hypersensitivity?
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Type III
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90% of patients with mixed
cryoglobulinemia are infected with ____? • What is found in cryoprecipitates? • _____ is found in cutaneous vasculitic lesions • _________ improves viremia and cryoglobulins |
Hepatitis C Virus
Hepatitis C Virus Interferon therapy |
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CURRENT TREATMENT OF
CRYOGLOBULINEMIA What reduces viral load? __________ agents still used |
Reduce viral load with interferon-α
Immunosuppressive still used |
