A case of IgG4-related kidney disease complicated by eosinophilic lung disease

Abstract

IgG4-related disease is a systemic chronic inflammatory disorder characterized by a high blood level of IgG4 and the organ injuries by marked infiltration of IgG4-positive plasma cells and fibrosis. A 71-year-old male was hospitalized for a cough, malaise and anorexia. IgG4-related disease was suspected due to marked elevation of the serum IgG4 level. However, on lung biopsy, only eosinophil infiltration was demonstrated with no plasma cell infiltration. Otherwise abdominal contrastenhanced CT showed mild enlargement of the bilateral kidneys and many differed contrasted areas and FDG PET-CT. Moreover, renal biopsy specimens showed typical tubulointerstitial nephritis with a large number of IgG4-positive plasma cells infiltration (the IgG4/IgG-positive cell rate, 89 %) and fibrosis. We diagnosed this patient as typical IgG4-related kidney disease. He was treated by the moderate dose of prednisolone (0.8 mg/kg/day) alone, and showed prompt response in the clinical condition, and both the lung and kidney lesions. In this case, it was useful for diagnosis of IgG4-related diseases to evaluate an image such as abdominal contrast-enhanced CT and FDG PET-CT. Our case might be one of the possible patterns of IgG4-related lung diseases. In addition, we thought that there might be an association between hypereosinophilia and IgG4-related kidney disease.

Case Report: IgG4-related kidney disease complicated by interstitial pneumonia

“…6 There have only been a few reports on the coexistence of interstitial pneumonia and IgG4-related kidney disease. 7 In this report, we describe the case of a patient who presented with interstitial pneumonia and was diagnosed with IgG4-tubulointerstitial nephritis and IgG4related kidney disease. Treatment with prednisolone was initiated soon after diagnosis and the patient responded well.…”

Case Report: IgG4-related kidney disease complicated by interstitial pneumonia

“… 6 There have only been a few reports on the coexistence of interstitial pneumonia and IgG4-related kidney disease. 7 In this report, we describe the case of a patient who presented with interstitial pneumonia and was diagnosed with IgG4-tubulointerstitial nephritis and IgG4-related kidney disease. Treatment with prednisolone was initiated soon after diagnosis and the patient responded well.…”

IgG4-related nephritis and interstitial pulmonary disease complicated by invasive pulmonary fungal infection: a case report

“…Patients with IgG4-RKD have an increased risk of infection than general population. IgG4-TIN can be accompanied by eosinophilic lung disease [ 12 ] and pneumonia [ 13 ]. Here, we present a case of IgG4-RKD and lung interstitial lesions who developed invasive pulmonary fungal infection (IPFI) during treatment of glucocorticoid combined with immunosuppressive agents.…”