Renal involvement in idiopathic hypereosinophic syndrome

Abstract

The hypereosinophilic syndromes (HESs) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. In idiopathic HES, the underlying cause of hypereosinophilia (HE) remains unknown despite thorough aetiological work-up. Kidney disease is thought to be rare in HES. Renal manifestations described include eosinophilic interstitial nephritis, various types of glomerulopathies, thrombotic microangiopathy (TMA) and electrolyte disturbances. The diagnosis must be made in time, because a recovery of renal function can be obtained if treatment is initiated promptly.

Membranous Nephropathy Associated with Atheroembolism

“…As shown in our case, eosinophilia and hypocomplementemia frequently occur during the acute phase of an atheroembolism, thereby showing immunological activation at the surface of the exposed emboli [10]. Approximately 20% of idiopathic hypereosinophilic syndrome (HES) patients develop proteinuria and hypertension, and renal manifestations include eosinophilic nephritis, MN, crescentic glomerulonephritis and immunotactoid nephritis, with or without immune deposits [11]. In both previous cases of MN associated with idiopathic HES, proteinuria, and eosinophilia greatly improved with corticosteroid treatment, as in our case in which the degree of proteinuria was closely associated with the eosinophil count [12,13] The mechanisms underlying renal involvement of HES were not well described, but were thought to be the same as those implicated in tissue damage of other organs: activated eosinophils release a number of cytopathic substances especially eosinophil granule major basic protein 1, eosinophil peroxidase, and other mediators [14].…”

Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

“…Hypereosinophilic syndrome (HES) is a disease characterized by an eosinophil count of over 1500 eosinophils/mm³ on at least two different occasions, with evidence of tissue hypereosinophilia or organ damage associated with hypereosinophilia (1,2). It is generally diagnosed by exclusion, with an estimated prevalence of 0.36 to 6.3 patients per 100 000 population.…”

“…It is generally diagnosed by exclusion, with an estimated prevalence of 0.36 to 6.3 patients per 100 000 population. It usually occurs between the ages of 20 and 50 and is more common among men (1,2). The disease may be primary, secondary, or idiopathic (1,2).…”

“…It usually occurs between the ages of 20 and 50 and is more common among men (1,2). The disease may be primary, secondary, or idiopathic (1,2). Secondary causes include parasitic or fungal infections, neoplasms, blood diseases, allergic diseases, and autoimmune diseases.…”

“…Skin, nervous system, lungs, and the heart are organs most often involved. Heart disorder is the main cause of death (1,2). While a case has been reported in which the kidney is the first organ to be involved (3), renal involvement is generally rare, while the disease itself being uncommon (2).…”