Table 1.
ORGAN SYSTEMS AFFECTED IN HES AND EGPA | ||
---|---|---|
HES | EGPA | |
Relative frequency: + Important cause of morbidity and mortality. eg, eosinophilic (necrotizing) myocarditis, mural thrombus formation, endomyocardial fibrosis |
Relative frequency: + eg, eosinophilic cardiomyopathy, endomyocardial fibrosis, coronary vasculitis, pericarditis |
|
Relative frequency: +++ Most common clinical manifestation of L-HES. eg, eczema, urticaria, erythematous papules or plaques, erythroderma, mucosal ulceration (M-HES), purpura |
Relative frequency: ++ eg, urticaria, purpura |
|
Relative frequency: ++ eg, eosinophilic gastrointestinal involvement, cholangitis, hepatitis |
Relative frequency: + eg, eosinophilic gastrointestinal involvement, ischemia, perforation (vasculitic involvement) |
|
Relative frequency: + Presence suggestive of underlying myeloid disease/neoplasm. eg, anemia, thrombocytopenia, splenomegaly |
Relative frequency: (+) (unusual) | |
Relative frequency: (+) (rarely reported in HES) eg, interstitial nephritis, eosinophilic cystitis |
Relative frequency: + More common in ANCA-positive patients who have vasculitic manifestations eg, glomerulonephritis, interstitial nephritis |
|
Relative frequency: + to ++ eg, cerebral vessel thrombosis, stroke (embolism from intracardiac thrombus), encephalopathy, peripheral neuropathy |
Relative frequency: ++ eg, peripheral neuropathy, mononeuritis multiplex, stroke |
|
Relative frequency: ++ More common in I-HES and M-HES. eg, asthma, interstitial pulmonary infiltrates, eosinophilic bronchitis, pleural effusion |
Relative frequency: ++++ (almost all patients with EGPA) eg, asthma, interstitial pulmonary infiltrates, alveolar hemorrhage |
|
Relative frequency: + eg, arthritis, tenosynovitis, fasciitis |
Relative frequency: + to ++ eg, arthralgia, arthritis |
|
Relative frequency: + to ++ eg, chronic rhinosinusitis |
Relative frequency: +++ Nasal polyps are common in EGPA. eg, chronic rhinosinusitis with or without polyposis |
|
Relative frequency: + Hypercoagulability and/or endovascular damage contribute to vascular events/complications. eg, arterial or venous thrombosis, microvascular damage, digital gangrene |
Relative frequency: +++ Hypercoagulability and/or small vessel vasculitis contribute to vascular events/complications (depending on affected organ system) eg, arterial or venous thrombosis, necrotizing vasculitis |
Relative frequencies are based on figures reported in the published literature or estimated based on the clinical observations of the authors: +, 0–<25% of patients; ++, ≥25–<50%; +++ ≥50–<75%; ++++ ≥75–100%.
ANCA, antineutrophil cytoplasmic antibody; EGPA, eosinophilic granulomatosis with polyangiitis; HES, hypereosinophilic syndrome; I-HES, idiopathic HES; L-HES, lymphocyte-variant HES; M-HES, myeloid/lymphoid neoplasm HES.