History
As vasculitides are systemic processes, a comprehensive history and full review of systems is necessary.
Possible triggers include the following:
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Recent infections (upper respiratory tract infections, especially streptococcal; tuberculosis [TB])
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Vaccinations - Eg, an Italian study found evidence that the measles-mumps-rubella (MMR) vaccine increases the risk of Henoch-Schönlein purpura (odds ratio = 3.4) [28]
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New medications
Presenting features include the following:
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Skin – Nodules, which may be painful, erythematous, or ulcerated; purpura; petechiae; papulopustular lesions; erythema nodosum; livedo reticularis; Raynaud phenomenon; lower extremity swelling
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Recurrent oral and/or genital ulcerations that heal with scarring
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Nasal/sinus – Allergic rhinitis, nasal congestion, recurrent epistaxis, sinus pain, tearing
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Pulmonary – Chronic cough, hemoptysis, shortness of breath, stridor, wheezing, chest pain
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Musculoskeletal – Joint pain or swelling, myalgias, calf pain
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Renal – Hematuria, hypertension
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GI tract – Abdominal pain, hematochezia, vomiting, nausea
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Genitourinary – Testicular swelling and/or pain
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Vascular – extremity claudication, blood pressure difference between limbs, pre-syncope/fainting spells
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Neurological – Headache, seizure, focal neurological deficits (eg, stroke, cranial nerve palsy, vision loss, fine motor deficits, movement abnormalities), diffuse neurological deficits (eg, cognitive decline, poor school performance), mononeuropathy or polyneuropathy (eg, numbness, paresthesias, hand/foot drop, weakness), visual/auditory hallucinations, fluctuating/decreased level of consciousness/encephalopathy
Past medical history may include the following:
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Recurrent sinusitis and pneumonias
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Asthma and allergic rhinitis precede development of Churg-Strauss syndrome
Family history may include an increased risk of Kawasaki disease in siblings.
Clinical features common in Henoch-Schönlein purpura include the following:
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Palpable purpuric rash (must be present)
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Diffuse abdominal pain
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Arthritis (acute, any joint) or arthralgia
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Hematuria and/or proteinuria
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Lower limb swelling may be seen
Clinical features common in Kawasaki disease include the following:
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Fever, at least 5 days in duration, unresponsive to antibiotic therapy
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Polymorphous exanthem (nonvesicular)
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Perineal desquamation
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Changes in peripheral extremities (erythema and/or edema)
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Cervical lymphadenopathy, usually unilateral, larger than 1.5 cm
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Bilateral, non-purulent, conjunctivitis
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Changes of lips and oral cavity - Injection of oral and pharyngeal mucosa, fissured lips
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Irritability, arthralgia/arthritis
Physical Examination
General
Vital sign measurements should include 4-limb blood pressure measurement to look for a difference of more than 10 mm Hg between limbs.
Head and Neck
Funduscopy and ophthalmologic assessment for conjunctivitis, uveitis, scleritis and/or retinal vasculitis, and optic neuritis is indicated. Orbital inflammation (lid edema/erythema, proptosis) may be seen in granulomatosis with polyangiitis and microscopic polyangiitis. [29]
Inspection of nasal cavity for bleeding, ulceration, and septal perforation is indicated.
Other findings may include the following:
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Nasal bridge swelling, pain or saddle-nose deformity (See the image below.)
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Paranasal sinus tenderness
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Cervical lymphadenopathy
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Oral ulcers seen in Behçet disease
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Otitis, mastoiditis, hearing loss
Respiratory
The following may be noted:
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Increased work of breathing, tachypnea, hypoxia
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Decreased breath sounds
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Stridor, wheeze
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Blood-tinged sputum
Cardiovascular
The following may be noted:
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Pulselessness, inequality of pulses
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Bruits over major arteries
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Increased capillary refill time
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Abnormal heart sounds, including gallop rhythm, muffled heart sounds, rub
Abdominal and genitourinary
Findings may include the following:
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Abdominal tenderness (focal or diffuse)
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Hepatomegaly, splenomegaly
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Blood on rectal examination
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Genital ulcers or scars in Behçet syndrome
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Testicular pain and/or swelling
Dermatologic
All patients with Henoch-Schönlein purpura have palpable purpuric rash, typically over buttocks and lower extremities.
Dermatologic manifestations of Kawasaki disease include polymorphous exanthem and red/swollen extremities.
Patients with polyarteritis nodosa have deep red tender subcutaneous nodules that can become ischemic and necrotic (see the images below).
Livedo reticularis may also be seen in childhood polyarteritis nodosa.
Musculoskeletal
These findings may include the following:
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Arthritis (joint swelling, effusions, decreased range of motion and/or stress pain)
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Muscle tenderness
Neurologic
These findings may include the following:
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Peripheral neuropathy, paresthesia
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Mononeuritis multiplex
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Cranial nerve palsies, vision loss, visual field defects
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Altered level of consciousness
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Focal neurological deficits, including stroke
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Diffuse neurological deficits
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Hallucinations, bizarre behavior
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Preferred sites of vascular involvement by selected vasculitides.
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Patient with Wegener granulomatosis and saddle-nose deformity.
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Tender erythematous nodules in cutaneous polyarteritis nodosa (PAN).
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Nodules on sole of foot in cutaneous polyarteritis nodosa (PAN).
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Necrotic lesions of polyarteritis nodosa (PAN).
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Chest radiography in Churg-Strauss syndrome (CSS) with pulmonary infiltrates.
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CT of sinuses in a patient with Wegener granulomatosis (WG) showing erosion and loss of sinus walls.
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CT chest in a patient with Churg-Strauss syndrome (CSS) showing multiple nodules.
Tables
Category |
Definition |
Localized |
Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms |
Early systemic |
Any, without organ-threatening or life-threatening disease |
Generalized |
Renal or other organ-threatening disease, serum creatinine >500 μmol/L (5.6 mg/dL) |
Severe |
Renal or other vital organ failure, serum creatinine >500 μmol/L (5.6 mg/dL) |
Refractory |
Progressive disease unresponsive to glucocorticoids and cyclophosphamide |
