Vasculitis Syndromes Flashcards
(43 cards)
What are vasculitis syndromes?
★ A clinicopathologic process of inflammation and damage to
blood vessels
★ Broad and heterogeneous group of syndromes
★ May be a primary disease or secondary component of another
disease
★ May be confined to a single organ or involve several organs
Etiology of Vasculitis syndromes
❖ Likely a number of factors are involved
➢ Genetic predisposition
➢ Environmental exposure
➢ Immune response to certain antigens
Eosinophilic Granulomatosis with
Polyangitis AKA
AKA “Churg-Strauss Syndrome”
Eosinophilic Granulomatosis with
Polyangitis etiology
❖ Exact cause unknown
❖ Allergic mechanism seems to be
directly involved
❖ T lymphocytes seem to help
maintain inflammation
❖ Any organ can be affected
Eosinophilic Granulomatosis with
Polyangitis: 3 phases
➢ Prodromal: persists for years. Allergic rhinitis,
nasal polyps, asthma or combination
➢ 2nd phase: peripheral blood and tissue
eosinophilia includes chronic eosinophilic
pneumonia and gastroenteritis
➢ 3rd phase: life threatening vasculitis. Systemic
symptoms fever, weight loss, malaise and fatigue
Eosinophilic Granulomatosis with
Polyangitis neurologic symtoms
mononeuritis multiplex occurs in 75% of patient
Eosinophilic Granulomatosis with
Polyangitis diagnosis
❖ Antineutrophil cytoplasmic Autoantibodies (ANCA) present in
40 % of cases
❖ Biopsy is dx tool of choice
❖ Treatment with systemic corticosteroids
❖ More severe cases may need immunosuppressant therapy with
methotrexate, cyclophosphamide or azathioprine
Temporal Arteritis s/s
❖ Headache-dominate symptom
➢ Jaw pain
➢ Fever
➢ Malaise
➢ PMR
➢ Blurred vision
50% of untreated patients
develop blindness with ____
Temporal Arteritis
Headache coarse in temporal arteritis
➢ Unilateral or bilateral superficial
➢ Located temporally about 50%
➢ Gradual onset with peak usually explosive
➢ Dull with stabbing pains
➢ Scalp tenderness cannot lay head on pillow
➢ Tender red temporal nodules may be present
➢ Worse at night
Temporal Arteritis diagnosis
➢ History and physical
➢ Erythrocyte sedimentation rate (ESR) often elevated
➢ Temporal artery biopsy
Temporal arteritis treatment
Prednisone 80 mg daily for 4 to 6 weeks
➢ Tocilizumab (ACTEMRA)–MCA against the interleukin-6
receptor
Eosinophilic Granulomatosis with
Polyangitis treatment
❖ Antineutrophil cytoplasmic Autoantibodies (ANCA) present in
40 % of cases
❖ Biopsy is dx tool of choice
❖ Treatment with systemic corticosteroids
❖ More severe cases may need immunosuppressant therapy with
methotrexate, cyclophosphamide or azathioprine
POLYARTERITIS NODOSA
❖ NECROTIZING VASCULITIS AFFECTING MEDIUM AND SMALL VESSELS
❖ MULTI ORGAN DISEASE SPARES THE LUNGS
❖ Disease usually of young adults
❖ Vague symptoms progress rapidly to fulminant illness
❖ Weakness, malaise, myalgias, new onset hypertension
❖ Most commonly affects kidney
POLYARTERITIS NODOSA treatment
➢ Combination of high dose prednisone and immunosupression
➢ Cyclophosphamide (Cytoxan) or Azathioprine (Imuran)
POLYARTERITIS NODOSA prognosis
➢ Untreated 5-10% mortality at 5 years
➢ Mortality from GI or cardiovascular
➢ Relapse in treated patients 10-20%
Granulomatosis With Polyangitis
❖ Granulomatous vasculitis of upper
and lower respiratory tracts
❖ Small vessel disease
❖ Uncommon disease rare in black pts
❖ 1:1 ratio male-to-female
❖ Can be seen at any age; mean age at
onset is 40
Granulomatosis With Polyangitis S/S
❖ Upper airway involvement in 95% of patients
➢ Paranasal sinus pain, purulent or bloody nasal discharge
➢ Nasal septal deviation resulting in saddle nose
➢ Serous otitis
➢ Subglottic stenosis
❖ Pulmonary involvement in 85-90%
➢ Cough, chest discomfort
➢ Hemoptysis, dyspnea
❖ Renal disease 77%
❖ Eye involvement 52%
❖ Skin lesions 46%
❖ Nervous system 23%
❖ Cardiac 8%
Granulomatosis With Polyangitis diagnosis
➢ Elevated ESR, WBC, mild elevated Rheumatoid Factor
➢ 90% will have a positive anti-proteinase-3 ANCA if disease is active, only 60%
if inactive
➢ Necrotizing granulomatous vasculitis on biopsy- pulmonary tissue offers
highest yield
➢ Upper airway and renal biopsy rarely show the vasculitis
Granulomatosis With
Polyangitis treatment
➢ Prior to onset of current treatment this was a universally fatal disease within
a few months of diagnoses
➢ Glucocorticoids improved symptoms but did not affect the ultimate outcome
➢ Cyclophosphamide (Cytoxin) changed outcome dramatically
Two phases of treatment induction and maintenance for Granulomatosis With
Polyangitis
❖ Induction-severe disease
➢ Prednisone 1 mg/kg for one month then taper over 9 months
➢ Cyclophosphamide 2 mg/kg/day orally for 3 months then reduce to 1.5 mg/kg
daily
➢ Blood counts and renal function should be monitored every 2 weeks
➢ Rituximab (Rituxan) 375 mg/m2 once a week for 4 weeks in combination with
prednisone found to be superior to cyclophosphamide in recurrent cases
❖ Induction-non severe cases
➢ Prednisone 1 mg/kg for one month then taper over 9 months
➢ Methotrexate (Trexall) 0.3mg/kg not to exceed 15 mg/week single dose if
tolerated well after 2 weeks may increase by 2.5 mg weekly to a dose not to
exceed 25 mg week
❖ Maintenance phase (after 3-6 months)
➢ Methotrexate at above doses
➢ Azathioprine (Imuran) 2 mg/kg day
Microscopic Polyangitis
❖ Nomenclature introduced in 1948 to recognize the presence of glomerulonephritis
in patients with polyarteritis nodosa
❖ 1992 was changed to connote a necrotizing vasculitis with few or no immune
complexes affecting capillaries and venules or arterioles (small vessel diseases)
❖ Glomerulonephritis is common
❖ Pulmonary capillaritis also occurs
❖ Absence of granulomatous inflammation differentiates it from Wegener’s
Microscopic Polyangiitis diagnosis
➢ Elevated ESR, anemia, leukocytosis and thrombocytosis
➢ ANCA (antineutrophil cytoplasmic antibodies) are present in 75% of patients
➢ Defined with histologic evidence on biopsy
Microscopic Polyangiitis treatment
➢ Same as Wegener’s
➢ 5 year survival rate is 74% with mortality from alveolar hemorrhage, GI,
cardiac or renal disease
➢ Relapse occurs in 34% of patients
