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27 Cards in this Set

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Name 5 etiologies of vasculitides
- Secondary Infection

- Immune complex

- Antiendothelial antibody

- Antineutrophil cytoplasmic antibody (ANCA)

- Direct infection: fungus, bacteria, Rickettsial
Profile of a Giant Cell (Temporal) Arteritis Patient
TEMPORAL ARTERITIS
3/5 Symptoms Needed to Dx
1) Age > 50

2) Headache or pain in head

3) Tenderness of temporal artery

4) Erythrocyte sedimentation rate (ESR) elevated (> 50 mm/hour)

5) Temporal artery biopsy: mononuclear infiltrate with granulomatous inflammation

*GCA is the most common vasculitis in Caucasians.
*Temporal biopsy remains the gold standard investigation.
Pathogenesis of Giant Cell (Temporal) Arteritis
GIANT CELL (TEMPORAL) ARTERITIS

1) Patchy inflammation (skip lesions) in arteries arising from the aorta:
-Superficial temporal
-Ophthalmic
-Vertebral

2) Due to occlusive vasculopathy rather than thrombosis––the proliferation of the intima results in loss of the lumen
Si/Sx:
Giant Cell(Temporal) Arteritis
Si/Sx: Giant Cell(Temporal) Arteritis
1) Scalp: Tenderness

2) Eye: Visual, motor, visual loss due to ischaemia

3)Acute phase Rx:
ESR, C-reactive Protein

4) Anemia: Normocytic, Normochromic

*Neuro-ophthalmic complications of GCA are common.
Profile of a Takayasu’s arteritis (Pulseless Disease) Patient
TAKAYASU'S ARTERITIS:
(Pulseless Disease)

1) <40 years of age;(contrast with temporal arteritis)

2) Female:male = 7:1

3) Stenosing lesions mid-subclavian arteries; granulomatous lesions of aorta and major branches = bruits and diminished or absent pulses

4) Histology similar to temporal arteritis
Additional Pathophysiology of Takayasu’s arteritis (Pulseless Disease) Patient
TAKAYASU'S ARTERITIS:(Pulseless Disease)

1) Ocular disturbances

2) Weakening of pulses in upper extremities

3) Fibrous thickening of aortic arch with narrowing of origins or more distal portions of great vessels arising from aorta
Patient Profile: Polyarteritis nodosa(classic polyarteritis nodosa)
POLYARTERITIS NODOSA(classic polyarteritis nodosa)

1) Peak age 40-60;

2) Weight loss

3) Myalgia

4) Skin: palpable purpura

5) Heart: pericarditis, infarct

6) Gastrointestinal: abdominal pain, infarction, hemorrhage

7) Histopathology: necrotizing vascular lesions; nodules with uninvolved spaces between lesions
Symptoms of Classic Polyarteritis nodosa
CLASSIC POLYARTERITIS NODOSA:

1) Weight loss > 4 kg

2) Livedo reticularis : skin rash

3) Testicular pain/tenderness

4) Myalgias

5) mono- polyneuropathy

6) Diastolic BP > 90

7) Elevated BUN and cretonne
HBsAg or antibody
Profile/diagnosis of apatient with
Kawasaki Disease(mucocutaneous lymph node syndrome)
KAWASAKI DISEASE:

1) Acute vasculitis of infancy and early childhood that spontaneously resolves

2) Most common vasculitis of childhood after Henoch-Schonlein purpura
- 80% of patients < 5 years of age
-Rare during first months of life

3) Does not occur in adults

4) Diagnosis based on clinical criteria b/c there is no diagnostic lab test
Pathophysiology of
Kawasaki Disease(mucocutaneous lymph node syndrome)
KAWASAKI DISEASE:

1) Permanent damage to coronary arteries occurs in 20-25% of untreated children
Clinical Features Kawasaki Disease
mucocutaneous lymph node syndrome)
Si/Sx Kawasaki:

1) > 5 days spiking fever

2) Skin rash: erythema and edema of palms and soles.

3) Lips and oral mucosa: cracked lips; strawberry tongue

4) Cervical lymphadenopathy

5) Conjunctivitis

6) Coronary artery aneurysms

7) Myocarditis
Etiology of Kawasaki Disease
mucocutaneous lymph node syndrome)
KAWASAKI DISEASE:

1) Unknown

2) Spread resembles viral or bacterial infection
Treatment of Kawasaki Disease(mucocutaneous lymph node syndrome)
Tx KAWASAKI DISEASE:
Aspirin + Immunoglobulin

1) Intravenous g-globulin + aspirin within the first 10 days of fever reduces risk of coronary artery damage

2) Goal is to minimize coronary aneurysms.

3) Aneurysms arise during acute phase, and can resolve, but long term follow-up needed.
Clinical Appearance
Leukocytoclastic Vasculitis
Clinical Appearance
Leukocytoclastic Vasculitis:

1) Nonblanching lesions first appear as purpuric macules

2) Symmetrically affect legs and ankles. Lesions evolve to papules (usually 3 to 6 mm in diameter with sharp margins)

3) May subsequently evolve to vesicles, nodules, ulcerations
HISTOLOGY
Leukocytoclastic Vasculitis
HISTOLOGY
Leukocytoclastic Vasculitis

1) IgM, IgG along postcapillary venules

2) Complement fixation

3) Neutrophils / lysosomal enzymes

4) Local destruction, leakage of fluid
Profile:
Leukocytoclastic Vasculitis Patient
Profile Leukocytoclastic Vasculitis Patient:

1) 60 year-old diabetic woman
2) Developed multiple violaceous grouped papules over the tops of the feet and lower legs over the preceding week.

3) Antistreptolysin titre was 240 iu/ml

4) Erythrocyte sedimentation rate was 21 mm/hour

5) Fasting blood sugar level was 270 mg%.

6) Itching and burning over the affected areas.

7)The lesions gradually progressed to involve the upper legs.

8) Symmetric discrete and confluent nonblanching violaceous papules
Etiology: Leukocytoclastic Vasculitis
1) Viral hepatitis (especially B and C)
2) Autoimmune (systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis)
3) Streptococci, staph, and other infections
4) Cryoglobulins
5) Ulcerative colitis
6) Lymphoproliferative disease (especially hairy cell leukemia)
7) Idiopathic
8) Thiazides, phenothiazines
9) Iodides, immune serum (from blood transfusions and other blood products)
10) Sulfa drugs, penicillin, and other antibiotics
Pathophysiology:
ANCA-associated vasculitides
Pathophysiology of ANCA-associated vasculitides

1) Systemic autoimmune diseases

2) Interplay of autoimmunity with environmental and genetic factors

3) Passive transfer of ANCA is sufficient to induce disease

*How are the autoantibodies to neutrophil antigens triggered?
Si/Sx:
Microscopic
Polyarteritis Nodosa
Si/Sx of Microscopic
Polyarteritis Nodosa:

1) Palpable purpura

2) Weight loss

3) Muscle and joint pains

4) Hypertension

5) Malaise

6) Multiple organ systems

7) Minority have HBsAg or HBsAb
Define P-ANCA
Name P-ANCA (+) Syndromes
P-ANCA: Perinuclear staining Antibodies directed against neutrophil protein myeloperoxidase (MPO).

P-Anca (+) Syndromes:
-Microscopic polyarteritis nodosa
-Churg-Strauss syndrome.
Define C-ANCA
Name C-ANCA (+) Syndromes
C-ANCA: Cytoplasmic granular staining directed against proteinase 3 (PR3)

C-ANCA (+):
Wegner's granulomatosis.
Si/Sx: Wegener’s Granulomatosis
Si/Sx Wegener's Gran:
1) Oral ulcers

2) Purulent nasal discharge

3) Chest x-ray showing nodules/infiltrates

4) Microhematuria or red cell casts

5) Granulomatous inflammation on biopsy
Stages of P-ANCA (+) Churg-Strauss
Stages of Churg-Strauss

Stage 1:
-Rhinitis; nasal polyps;
-Asthma (begins mean age 35)

Stage 2:
-Eosinophilic pneumonia; gastroenteritis;
-Peripheral eosinophilia


Stage 3:
-Systemic vasculitis which may occur up to 30 years after initial symptoms
Profile, Si/Sx: Henoch-Schonlein Purpura
Profile/Si,Sx H-S Purpura:

1) Children > adults

2) Preceded by URI

3) Palpable purpura

4) Glomerulonephritis

5) GI bleeding

6) IgA deposition
Si/Sx: Cryoglobulinemic Vasculitis
Si/Sx: Cryoglobulinemic Vasculitis

1) Rare systemic vasculitis

2) Most have glomerulonephritis

3) Cutaneous vasculitis

4) Deposits of IgG-IgM that precipitate in the cold
Profile, Si/Sx:
Buerger’s Disease
Thrombangiitis Obliterans
Profile, Si/Sx:
Buerger’s Disease
Thrombangiitis Obliterans

1) Distal extremities
Lungs, kidneys, brain, GI tract spared

2) Closely related to smoking

3) Traditionally males < 35 years old
Profile, Si/Sx:
Behcet’s Disease
Profile, Si/Sx:
Behcet’s Disease

1) Significant cause of blindness in Japan

2) Aphthous oral ulcers

3) Genital lesions

4) Eye inflammation

5) Vasculitis of nearly all size vessels and involves arteries and veins.

6) Waxes and wanes