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. Author manuscript; available in PMC: 2024 Jul 1.
Published in final edited form as: Mayo Clin Proc. 2023 Jul;98(7):1054–1070. doi: 10.1016/j.mayocp.2023.02.013

Table 1.

Organ systems affected in HES and EGPA6, 7, 916

ORGAN SYSTEMS AFFECTED IN HES AND EGPA
HES EGPA
graphic file with name nihms-1877852-t0002.jpg 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
graphic file with name nihms-1877852-t0003.jpg 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
graphic file with name nihms-1877852-t0004.jpg Relative frequency: ++
eg, eosinophilic gastrointestinal involvement, cholangitis, hepatitis
Relative frequency: +
eg, eosinophilic gastrointestinal involvement, ischemia, perforation (vasculitic involvement)
graphic file with name nihms-1877852-t0005.jpg Relative frequency: +
Presence suggestive of underlying myeloid disease/neoplasm.
eg, anemia, thrombocytopenia, splenomegaly
Relative frequency: (+) (unusual)
graphic file with name nihms-1877852-t0006.jpg 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
graphic file with name nihms-1877852-t0007.jpg Relative frequency: + to ++
eg, cerebral vessel thrombosis, stroke (embolism from intracardiac thrombus), encephalopathy, peripheral neuropathy
Relative frequency: ++
eg, peripheral neuropathy, mononeuritis multiplex, stroke
graphic file with name nihms-1877852-t0008.jpg 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
graphic file with name nihms-1877852-t0009.jpg Relative frequency: +
eg, arthritis, tenosynovitis, fasciitis
Relative frequency: + to ++
eg, arthralgia, arthritis
graphic file with name nihms-1877852-t0010.jpg Relative frequency: + to ++
eg, chronic rhinosinusitis
Relative frequency: +++
Nasal polyps are common in EGPA.
eg, chronic rhinosinusitis with or without polyposis
graphic file with name nihms-1877852-t0011.jpg 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.