Definition
A.Part of a group of disorders that result from inflammatory changes to walls of veins and arteries, causing damage to the mural structures that ultimately may cause tissue ischemia and necrosis.
B.Nomenclature is changing for the specific forms of vasculitis based on the Chapel Hill Consensus Conference (CHCC).
C.There are several main types of small vessel vasculitis.
1.Henöch–Schonlein purpura (IgA vasculitis).
2.Antineutrophil cytoplasmic antibodies (ANCA)-associated disorders.
a.Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis).
b.Microscopic polyangiitis.
i.Most common cause of pulmonary-renal syndromes.
ii.Does not cause chronic upper respiratory tract disease.
iii.Does not have granulomatous inflammation on biopsy.
c.Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome).
Incidence
A.Henöch–Schonlein purpura (IgA vasculitis).
1.90% of cases occur in pediatric population.
B.ANCA-associated disorders.
1.Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis).
a.12 cases in 1 million people annually.
b.Not gender specific.
c.Age of onset commonly fourth to fifth decades.
2.Microscopic polyangiitis.
a.Most common cause of pulmonary-renal syndromes.
3.Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome).
a.Mean age of onset is 40.
b.No gender specificity.
c.Possibility of genetic predisposition.
Pathogenesis
A.Vasculitis can be a primary pathology or can be secondary to another underlying disease process.
B.Direct injury to the vessel, infectious agents, or immune processes are known to cause vasculitis.
C.Secondary vascular injury may occur from physical agents such as cold and irradiation, mechanical injuries, and toxins.
D.Small vessel vasculitis may also be associated with ANCA.
1.Henöch–Schonlein purpura (IgA vasculitis): Associated with IgA subclass 1 deposition in vessel walls.
2.ANCA-associated disorders.
a.Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis).
i.Characterized by upper and lower respiratory tract disease and glomerulonephritis.
ii.Fatal if not treated.
b.Microscopic polyangiitis: Caused by necrotizing vasculitis.
c.Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome): Idiopathic in patients with asthma.
Predisposing Factors
A.Henöch–Schonlein purpura (IgA vasculitis): Recent infection such as group A streptococcus.
B.ANCA-associated disorders: Systemic diseases such as systemic lupus erythematosus, rheumatoid conditions, and polychondritis can predispose patients.
1.Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis).
2.Microscopic polyangiitis.
3.Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome).
Subjective Data
A.Common complaints/symptoms.
1.Small vessel vasculitis will often present with purpura, vesiculobullous lesions, urticaria, glomerulonephritis, alveolar hemorrhage, cutaneous extravascular necrotizing granulomas, splinter hemorrhages, uveitis, episcleritis, and scleritis.
a.Henöch–Schonlein purpura (IgA vasculitis).
i.Palpable purpura usually on lower extremities, arthralgias of knees and ankles, and hematuria.
b.ANCA-associated disorders.
i.Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis).
1)Upper respiratory tract: Nasal congestion, crusting, ulceration, bleeding, septal perforation, saddle nose,
sinusitis, otitis media, mastoiditis, gingival edema, stridor, subglottic stenosis.
2)Lower respiratory tract: Cough, dyspnea, hemoptysis.
3)Renal system: Hematuria, often not evident until disease is advanced (UTD).
4)Other systems: Arthritis, ocular manifestations such as proptosis, scleritis, episcleritis, conjunctivitis, skin lesions, neuropathy.
5)Deep vein thrombosis (DVT)s and pulmonary embolism (PE)s are also common.
ii.Microscopic polyangiitis.
1)Palpable purpura.
2)Ulcers.
3)Splinter hemorrhages.
4)Vesicular bullous lesions.
5)Interstitial lung fibrosis.
6)Pulmonary-renal syndromes.
iii.Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome).
1)Allergic rhinitis.
2)Asthma.
B.Review of systems.
1.Fever.
2.Fatigue.
3.Weight loss.
4.Arthralgias.
5.Respiratory symptoms (rhinorrhea, cough).
6.Skin changes.
7.Eye pain.
8.Eye redness.
9.Hematuria.
Physical Examination
A.Henöch–Schonlein purpura (IgA vasculitis).
1.Palpable purpura in lower extremities and buttock.
B.ANCA-associated disorders.
1.Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis).
a.Nasal examination will reveal the following.
i.Congestion.
ii.Crusting.
iii.Ulceration.
iv.Bleeding.
v.Septal perforation.
b.Saddle nose
deformity is a late finding.
c.Otitis media.
d.Proptosis.
e.Conjunctivitis.
f.Scleritis.
g.Episcleritis.
h.Signs of DVT.
i.Calf swelling.
ii.Erythema.
iii.Tenderness.
2.Microscopic polyangiitis.
a.Palpable purpura.
b.Ulcers.
c.Splinter hemorrhages.
d.Vesiculobullous lesions.
e.Pneumonitis.
3.Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome).
a.Skin and lung involvement.
i.Wheezing.
ii.Rhinitis.
iii.Macular rash.
iv.Urticaria.
v.Palpable purpura.
Diagnostic Tests
A.Henöch–Schonlein purpura (IgA vasculitis).
1.Biopsy will reveal leukocytoclastic vasculitis with IgA deposition.
B.ANCA-associated disorders.
1.Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis).
a.Leukocytosis, thrombocytosis, normocytic, normochromic anemia.
b.Elevated creatinine.
c.Elevated C-reactive protein (CRP) and elevated erythrocyte sedimentation rate (ESR).
d.Positive ANCA.
e.Red cell casts and proteinuria.
f.Lung biopsy more likely to show granulomas.
g.Chest CT.
2.Microscopic polyangiitis.
a.75% will be positive.
b.Microscopic hematuria, proteinuria, red blood cell casts.
c.Renal biopsy for necrotizing glomerulonephritis.
3.Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome).
a.Eosinophilia in peripheral blood smear.
b.Positive ANCA.
Differential Diagnosis
A.Other types of vasculitis.
B.Infection.
C.Malignancy.
D.Atherosclerosis.
E.Thromboembolic disease.
F.Congenital/hereditary disorders.
G.Hypercoagulable states.
H.Inflammatory disorders (you may want to consider making this a general differential diagnosis for all forms of vasculitis).
Evaluation and Management Plan
A.General plan.
1.Henöch–Schonlein purpura (IgA vasculitis).
a.Corticosteroids have been controversial and have not demonstrated reduction in long-term complications.
2.ANCA-associated disorders.
a.The disease process involves multiple systems. Treatment is symptomatic.
B.Patient/family teaching points.
1.Requires regular examinations and tests to monitor for complications of the disease process.
2.Take medications as indicated.
C.Pharmacotherapy.
1.Henöch–Schonlein purpura (IgA vasculitis).
a.Nonsteroidal anti-inflammatory drugs (NSAIDs) and Tylenol for treatment of pain; corticosteroids are reserved for refractory pain.