Limits...
IgG4-related Kidney Disease Mimicking Malignant Ureter Tumor

View Article: PubMed Central - PubMed

ABSTRACT

Immunoglobulin G4-related disease is a recently recognized systemic disease that can affect any organ or tissue in the body, including the kidneys. IgG4-related kidney disease (IgG4-RKD) is an important part of immunoglobulin G4-related disease. The most common renal manifestation of IgG4-RKD is tubulointerstitial nephritis and glomerular lesions. There, however, is few case of IgG4-RKD mimicking malignant ureter tumor leading to severe hydronephrosis. We herein report an unusual case of IgG4-RKD mimicking malignancy.

A 66-year-old Asian man presented to the nephrologist with soreness of loins, anorexia, and acute kidney injury in 2010. His renal function spontaneously improved after 2 weeks’ hemodialysis without systemic steroid therapy. Four years later, he presented to the urologist with severe left hydronephrosis because of marked thickness of the left ureter wall. As a ureteral malignancy could not be ruled out, laparoscopic nephroureterectomy was performed.IgG4-related kidney disease was confirmed by the histologic examination. Then, repeat laboratory test showed almost complete recovery of renal function after initiation of steroidal therapy.

This case highlights the rare possibility of IgG4-RKD mimicking malignant ureter tumor. Nephrologist and pathologists should be aware of the possibility that hydronephrosis with ureter obstruction may be involved in IgG4-RKD.

No MeSH data available.


Granular electron-dense deposits (arrow) are accumulated in the thickened tubular basement membrane. TC = tubular cell, PC = plasma cell (×100).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4998279&req=5

Figure 4: Granular electron-dense deposits (arrow) are accumulated in the thickened tubular basement membrane. TC = tubular cell, PC = plasma cell (×100).

Mentions: Histologic examination revealed lymphoplasmacytic infiltrate with storiform fibrosis in renal cortex and ureter wall. Infiltrating plasma and lymphocytes cells did not show significant cytologic atypia. Also, there was no evidence of malignancy in ureter wall. Immunohistochemically, the IgG4 positive plasma cells infiltrated exceeding 10 cells per 1 high-power field. More than 50% of plasma cells positive for IgG were positive for IgG4 (Figure 3). By electron microscopy, electron-dense deposits were seen in tubular basement membrane (Figure 4). Immunoglobulin immunofluorescence revealed strong accumulation in the tubular basement membrane and Bowman capsule (Figure 5). Based on pathologic results after surgery, we analyzed stored preoperative serum retrospectively. As a result, the patients had polyclonal gammopathy with elevated levels of serum IgG (1910 mg/dl), but serum IgE was normal (76 IU/mL). Hypocomplementemia was found. The serum level of IgG4 was also elevated (913 mg/dL). Finally, we diagnosed IgG4-related kidney disease with renal and ureter lesion according to a diagnostic criteria proposed in the literature.8 After the operation, the patient received steroidal therapy. Oral prednisolone at initial dose of 60 mg/day was administrated after surgery. Within 2 week, he had improvement in renal function. The serum levels of creatinine returned to 94 μmol/l three months after the initiation of therapy and the serum level of IgG4 returned to 53 mg/dL (normal value ≤135 mg/dL). The dose of prednisolone was gradually tapered and he has been treated with 10 mg daily since March 2015, without further symptoms. His renal function and IgG4 levels were normal during follow-up of 10 months.


IgG4-related Kidney Disease Mimicking Malignant Ureter Tumor
Granular electron-dense deposits (arrow) are accumulated in the thickened tubular basement membrane. TC = tubular cell, PC = plasma cell (×100).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4998279&req=5

Figure 4: Granular electron-dense deposits (arrow) are accumulated in the thickened tubular basement membrane. TC = tubular cell, PC = plasma cell (×100).
Mentions: Histologic examination revealed lymphoplasmacytic infiltrate with storiform fibrosis in renal cortex and ureter wall. Infiltrating plasma and lymphocytes cells did not show significant cytologic atypia. Also, there was no evidence of malignancy in ureter wall. Immunohistochemically, the IgG4 positive plasma cells infiltrated exceeding 10 cells per 1 high-power field. More than 50% of plasma cells positive for IgG were positive for IgG4 (Figure 3). By electron microscopy, electron-dense deposits were seen in tubular basement membrane (Figure 4). Immunoglobulin immunofluorescence revealed strong accumulation in the tubular basement membrane and Bowman capsule (Figure 5). Based on pathologic results after surgery, we analyzed stored preoperative serum retrospectively. As a result, the patients had polyclonal gammopathy with elevated levels of serum IgG (1910 mg/dl), but serum IgE was normal (76 IU/mL). Hypocomplementemia was found. The serum level of IgG4 was also elevated (913 mg/dL). Finally, we diagnosed IgG4-related kidney disease with renal and ureter lesion according to a diagnostic criteria proposed in the literature.8 After the operation, the patient received steroidal therapy. Oral prednisolone at initial dose of 60 mg/day was administrated after surgery. Within 2 week, he had improvement in renal function. The serum levels of creatinine returned to 94 μmol/l three months after the initiation of therapy and the serum level of IgG4 returned to 53 mg/dL (normal value ≤135 mg/dL). The dose of prednisolone was gradually tapered and he has been treated with 10 mg daily since March 2015, without further symptoms. His renal function and IgG4 levels were normal during follow-up of 10 months.

View Article: PubMed Central - PubMed

ABSTRACT

Immunoglobulin G4-related disease is a recently recognized systemic disease that can affect any organ or tissue in the body, including the kidneys. IgG4-related kidney disease (IgG4-RKD) is an important part of immunoglobulin G4-related disease. The most common renal manifestation of IgG4-RKD is tubulointerstitial nephritis and glomerular lesions. There, however, is few case of IgG4-RKD mimicking malignant ureter tumor leading to severe hydronephrosis. We herein report an unusual case of IgG4-RKD mimicking malignancy.

A 66-year-old Asian man presented to the nephrologist with soreness of loins, anorexia, and acute kidney injury in 2010. His renal function spontaneously improved after 2 weeks’ hemodialysis without systemic steroid therapy. Four years later, he presented to the urologist with severe left hydronephrosis because of marked thickness of the left ureter wall. As a ureteral malignancy could not be ruled out, laparoscopic nephroureterectomy was performed.IgG4-related kidney disease was confirmed by the histologic examination. Then, repeat laboratory test showed almost complete recovery of renal function after initiation of steroidal therapy.

This case highlights the rare possibility of IgG4-RKD mimicking malignant ureter tumor. Nephrologist and pathologists should be aware of the possibility that hydronephrosis with ureter obstruction may be involved in IgG4-RKD.

No MeSH data available.