Idiopathic Hypereosinophilic Syndrome Presenting as IgA Nephropathy with Nephrotic Range Proteinuria

Abstract

Idiopathic hypereosinophilic syndrome (IHES) is a disorder characterized by increased eosinophil count (eosinophilia) along with organ dysfunction secondary to organ infiltration of eosinophils and release of inflammatory markers [1][2][3][4], with no obvious cause for eosinophilia. The onset of symptoms is insidious in most of the cases and eosinophilia is detected incidentally. However, in others, the initial manifestations are severe and life-threatening due to the rapid evolution of cardiac or neurologic complications [5]. Renal involvement is rarely reported [6] in IHES. Herein we reported a case of IHES with predominant renal involvement as nephrotic syndrome with focal necrotizing IgA nephropathy.Keywords: Idiopathic Hypereosinophilic Syndrome; Eosinophilia; Renal Involvement; Nephrotic Syndrome Case ReportA 50-year old male presented with a 3-week history of a non-productive cough, associated with intermittent low grade fever, periorbital puffiness and lower extremity edema. Patient denied any history of breathlessness, chest-pain, hemoptysis, orthopnea, paroxysmal nocturnal dyspnea, drop in urine output, skin rash or arthritis. His past history was insignificant and he denied any history of drug intake.Hisexamination revealed mild pallor, with periorbitalpuffiness, lower extremity pitting edema and mild splenomegaly. He had hemoglobin of 11 g/dL with a total leucocyte count of 9.2 × 10 9 /cumm and an ESR of 23 mmhr. His differential count included 46% polymorphs 11% lymphocytes and 41% eosinophils with AEC count of 3800 which repeatedly was high and ranged from 3800 to 5500. A peripheral blood film showed features of normochromic normocytic anaemia with no abnormal cells. His serum creatinine was 1.7 mg/dL with a creatinine clearance of 44 ml/min. Liver functions showed hypoporoteinemia with hypoalbuminemia. He had an active urinary sedimentand urine which showed presence of eosinophils on wright's staining. 24 hours urinary protein was 5.04 g/24hours which was reproduced twice. Repeated stool examination for the presence of ova/parasite was negative.Radiograph of the chest was normal. High resolution computerized tomography of the chest did not reveal any abnormality. His electrocardiograph and echocardiography were normal. Ultrasonography of abdomen was normal as well.Patient was investigated further to look for eosinophilic infiltration of other organ systems in view of persistant hypereosinophilia.Patient was subjected to bronchoscopy to obtain bronchoalveolar lavage for eosinophils which revealed 62% eosinophils. Bone marrow aspiration and biopsy revealed 56% eosinophils with no dysplasia and blasts. p-ANCA andc-ANCA were within normal limits. Serum tryptase levels were within normal limits.Patient was subjected to percutaneous renal biopsy to look for the etiology of nephrotic range proteinuria. Renal biopsy revealed, marked interstitial edema and patchy inflammation including aggregates of eosinophils admixed with mononuclear cells and occasional neutrophils. An eosinophilic infiltrate wa...

Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

“…However, it is thought to be similar to histologic involvement by HES on other organs by mass effect due to the proportion of eosinophils and eosinophil cytotoxicity (2). In fact, these reports are characterized by eosinophilic infiltration in renal biopsy, which does not occur in our patient (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). While focal segmental glomerulosclerosis and HES may be two coexisting diseases-yet unrelated in our patient-the low proportion of cases reported in the literature of glomerular involvement of HES, compels us to consider the possibility of physiopathological mechanisms not yet known may account for such coexistence.…”

“…Kidney involvement is related to vascular disorder, interstitial nephritis, electrolyte disturbances, CharcotLeyden crystals or glomerular diseases (2). Few cases of HES with glomerular lesions have been reported in medical literature (4)(5)(6)(7)(8)(9)(10)(11). We describe a case of HES with nephrotic syndrome secondary to focal segmental glomerulosclerosis.…”