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2012 ; 1
(2
): 117-122
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Systemic and rapidly progressive light-chain deposition disease initially
presenting as tubulointerstitial nephritis
#MMPMID28509073
Takahashi S
; Soma J
; Nakaya I
; Yahata M
; Sakuma T
; Yaegashi H
; Sato A
; Wano M
; Sato H
CEN Case Rep
2012[Nov]; 1
(2
): 117-122
PMID28509073
show ga
A 42-year-old woman was admitted to a hospital after first-time detection of
proteinuria and hematuria during a routine medical check-up. Because her serum
creatinine level had rapidly increased from 0.9 to 3.2 mg/dl since measurement
3 months earlier, she was referred to our hospital. Renal biopsy revealed
extensive tubular atrophy and interstitial fibrosis with mild leukocyte
infiltration. Glomeruli showed minimal changes, and no immunoglobulin or
complement deposition was observed by immunofluorescence. Oral prednisolone was
commenced under the diagnosis of chronic tubulointerstitial nephritis, and she
discharged once. However, its effects were transient; her renal function
deteriorated rapidly and hemodialysis was initiated 5 months after her initial
check-up. On readmission, urinary Bence-Jones protein ?-type was detected, and
examination of bone marrow led to a diagnosis of Bence-Jones ?-type multiple
myeloma. Light-chain staining using a renal biopsy specimen obtained 2 months
earlier showed ?-light-chain deposition on tubular basement membranes but not
glomeruli. Despite undergoing chemotherapy with vincristine, doxirubicin, and
dexamethasone, the patient died suddenly from a cardiac arrhythmia. Autopsy
showed ?-light-chain deposition in the heart, thyroid, liver, lungs, spleen, and
ovaries. Congo red staining yielded negative results. Typical light-chain
deposition disease (LCDD) characterized by nodular glomerulosclerosis was
observed in the kidneys. This case demonstrates that tubulointerstitial nephritis
can be an early pathological variant of LCDD, which may be followed by
accelerated and massive light-chain deposition in glomeruli.