Page 829 - Clinical Immunology_ Principles and Practice ( PDFDrive )

CHaPtEr 58 Small- and Medium-Vessel Primary Vasculitis 801

TABLE 58.3 assessing Disease activity in Vasculitis
System
assessed BVaS BVaS WG PVaS Comments
General Myalgia, arthralgia/ arthritis, Arthritis, fever Myalgia, arthralgia/ arthritis, These are typical features in many forms
fever, weight loss fever, weight loss of systemic vasculitis.
Skin Infarct, purpura, skin ulcer, Purpura, skin ulcer, Polymorphous exanthema, Skin is a common organ involved in most
gangrene gangrene livedo, panniculitis, forms of small and medium vessel
purpura, skin nodules, vasculitis. Skin manifestations in
infarct, ulcer, gangrene, ANCA-associated vasculitides tend to be
other skin vasculitis less serious compared with
manifestations in kidneys, lungs, and
upper airways.
For skin vasculitis in children the
manifestations are more diverse.
Mucous Mouth ulcers, genital Mouth ulcer, conjunctivitis/ Mouth ulcers, genital Eye involvement is most common in GPA
Membrane/ ulcers, adnexal episcleritis, retro-orbital ulcers, adnexal and less often seen in MPA or EGPA.
eyes inflammation, proptosis, mass, uveitis, scleritis, inflammation, proptosis,
red eye, (epi)scleritis, retinal exudates red eye (epi)scleritis,
conjunctivitis, blepharitis, conjunctivitis, blepharitis,
keratitis, blurred vision, keratitis, blurred vision,
visual loss, uveitis, retinal visual loss, uveitis, retinal
vasculitis vasculitis
Ear, nose, Bloody nasal discharge, Blood nasal discharge, Blood nasal discharge, ENT manifestations are most common in
and throat paranasal sinus paranasal sinus paranasal sinus GPA and rarely seen in MPA or EGPA.
involvement, subglottic involvement, swollen involvement, subglottic However, EGPA is often characterized
stenosis, conductive salivary glands, subglottic stenosis, conductive by the presence of nasal polyps, which
hearing loss, inflammation, conductive hearing loss, are inflammatory in nature.
sensorineural hearing hearing loss, sensorineural sensorineural hearing
loss hearing loss loss
Chest Wheezing, nodules or Nodules or cavities, Wheezing, nodules or The chest is commonly affected in all
cavities, pleural effusion, pleurisy, infiltrates, cavities, pleural effusion, three forms of ANCA vasculitis.
infiltrates, endobronchial endobronchial changes, infiltrates, endobronchial Wheezing is a common feature of EGPA
changes, massive alveolar hemorrhage changes, massive as are infiltrates. In contrast, infiltrates,
hemoptysis, respiratory hemoptysis, respiratory hemoptysis, respiratory nodules, and endobronchial disease
failure failure failure dominate in GPA. Massive hemoptysis
can occur in patients with GPA or MPA
and less frequently in patients with
EGPA, but may also be a typical feature
with GBM (glomerular basement
membrane) disease.
Cardiovascular Loss of pulses, ischemic Pericarditis Loss of pulses, bruits, Cardiovascular manifestations are most
system cardiac pain, blood pressure widely recognized in KD, which is not
cardiomyopathy, discrepancy, claudication, particularly well covered by PVAS.
congestive cardiac failure, ischemic cardiac pain, Although CVD manifestations do occur
valvular heart disease, cardiomyopathy, in small- and medium-vessel vasculitis,
pericarditis congestive cardiac failure, they are more typically seen in large
valvular heart disease, vessel diseases, such as Takayasu
pericarditis arteritis.
Abdominal Ischemic abdominal pain, Mesenteric ischemia Abdominal pain, peritonitis, Gut involvement is more typical in
system peritonitis, bloody bloody diarrhea, bowel medium-vessel vasculitis, especially
diarrhea ischemia polyarteritis nodosa, but is well
recognized in patients with GPA
especially with colitis giving rise to
bloody diarrhea.
Renal system Hypertension, proteinuria, Hematuria, red cell casts, or Hypertension, proteinuria, Renal involvement in small-vessel
hematuria, impaired renal glomerulonephritis, hematuria, impaired renal vasculitis is one of the major
function, deterioration in deterioration in renal function, deterioration in manifestations leading to organ failure
renal function function renal function and death and should be carefully
assessed. Renal involvement in
medium-vessel vasculitis is much less
common and takes the form of infarction
of segments of the kidney leading to
hematuria and hypertension with
consequent impairment of renal function.
Nervous Headache, meningitis, Meningitis, stroke, cord Headache, meningitis, Neurological involvement is a common
system organic confusion, involvement, cranial nerve organic confusion, feature in small- and medium-vessel
seizures, stroke, cord lesion, sensory or motor seizures, stroke, cord vasculitis; often it does not lead to
involvement, cranial neuropathies involvement, cranial immediate loss of life. Strokes are less
nerve, lesion, sensory or nerve, lesion, sensory or common, whereas peripheral
motor neuropathies motor neuropathies neuropathies are more common and can
cause long-term disability.