0 mg/dL) levels were high, although other IgG subtypes were norma

0 mg/dL) levels were high, although other IgG subtypes were normal. Serum immunofixation did not demonstrate M protein, and the level of serum soluble IL-2 receptor was normal. The serum levels of κ (20.6 mg/dL) and λ (18.5 mg/dL) free light chains and the κ/ λ ratio (1.11) were also normal. The patient also did not have any donor specific antigens. A contrast-enhanced CT scan revealed a non-enhanced mass at the renal hilum

and some contrast defect areas in the renal cortex and diffuse marked enlargement of the graft, although no lymph node swelling was observed (Fig. 2A). An MRI also showed a hilum mass lesion with high intensity on T2-weighted images check details and low intensity on diffusion-weighted images. A PET-CT scan only detected a light integrated

mass of the hilum. Based on these findings, the patient was suspected of having IgG4-RKD. As the renal function of the patient was stable at that time, a no-treatment follow-up strategy was considered appropriate. However, her renal function deteriorated gradually and the serum Palbociclib chemical structure IgG4 level remained high (>400 mg/dL). In November 2012, the patient’s serum creatinine level had increased to 1.56 mg/dL. A biopsy was therefore carried out that showed almost the same findings as the biopsy 2 years after transplantation, although some severe fibrotic lesions and infiltration of IgG4-positive plasma cells were observed directly under the renal capsule. Because of the deterioration in renal function, the methylprednisolone dose was increased to 16 mg/day. Three months after this increase in steroid dose, the hilum mass disappeared on a CT scan (Fig. 2B), but cytomegalovirus antigenmia, JC virus viruria and viraemia screening became positive. An over-immunosuppression state was therefore suspected, and the methylprednisolone dose was decreased to 8 mg/day and mycophenolate mofetil changed to mizoribine. 4-Aminobutyrate aminotransferase Five months after the initial increase in steroid

dose, a follow-up biopsy in May 2013 showed that plasma cell infiltration in the renal interstitium had decreased markedly, although focal and segmental severe interstitial fibrosis and tubular atrophy with IgG4-positive plasma cells were observed (Fig. 3). Serum IgG4 levels decreased immediately after the increase in steroid dose and remained at <100 mg/dL thereafter. The patient's serum creatinine level also remained stable at around 1.6 mg/dL. The clinical course of the patient is shown in Figure 4. IgG4-RKD usually manifests as plasma cell-rich tubulointerstitial nephritis (TIN), although its clinicopathological features are not well described. Raissan et al. showed that most patients with overt IgG4-RKD had acute or progressive chronic renal failure, involvement of other organ systems, radiographic abnormalities such as small peripheral low-attenuation cortical nodules or diffuse marked enlargement of the kidneys, and elevated IgG4 serum levels (>135 mg/dL).

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