Tubulointerstitial Nephritis in a Patient With Probable Autoimmune Lymphoproliferative Syndrome

Mia Glerup; Troels Herlin; Søren Rittig; Kirsten Grønbæk; Marianne Hokland; Henrik Hasle

doi:10.1097/mph.0b013e31828ac9fe

Tubulointerstitial Nephritis in a Patient With Probable Autoimmune Lymphoproliferative Syndrome

Mia Glerup, Troels Herlin, Søren Rittig, Kirsten Grønbæk, Marianne Hokland, Henrik Hasle

Department of Clinical Medicine

3 Citations (Scopus)

Abstract

Autoimmune lymphoproliferative syndrome (ALPS) is caused by a nonmalignant defective Fas-mediated apoptosis. The main clinical manifestations are chronic lymphadenopathy, splenomegaly, and autoimmune cytopenia. Most patients with ALPS have a FAS germline mutation. ALPS has occasionally been associated with glomerulonephritis and we present the first report of tubulointerstitial nephritis associated with probable ALPS. A 5-year-old girl presented with fever, vomiting, hypertension, and azotemia. No autoantibodies, viral, or streptococcal antibodies were detected. A renal biopsy showed small-vessel vasculitis with normal glomeruli and inflammation in the interstitium. The patient responded to prednisolone treatment and obtained a full renal recovery. Symptoms of connective tissue disorder supervened and after the development of more pronounced splenomegaly, a diagnosis of ALPS was confirmed.

Original language	English
Journal	Journal of Pediatric Hematology/Oncology
Volume	35
Issue number	5
Pages (from-to)	e187-e189
Number of pages	3
ISSN	1077-4114
DOIs	https://doi.org/10.1097/mph.0b013e31828ac9fe
Publication status	Published - Jul 2013

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title = "Tubulointerstitial Nephritis in a Patient With Probable Autoimmune Lymphoproliferative Syndrome",

abstract = "Autoimmune lymphoproliferative syndrome (ALPS) is caused by a nonmalignant defective Fas-mediated apoptosis. The main clinical manifestations are chronic lymphadenopathy, splenomegaly, and autoimmune cytopenia. Most patients with ALPS have a FAS germline mutation. ALPS has occasionally been associated with glomerulonephritis and we present the first report of tubulointerstitial nephritis associated with probable ALPS. A 5-year-old girl presented with fever, vomiting, hypertension, and azotemia. No autoantibodies, viral, or streptococcal antibodies were detected. A renal biopsy showed small-vessel vasculitis with normal glomeruli and inflammation in the interstitium. The patient responded to prednisolone treatment and obtained a full renal recovery. Symptoms of connective tissue disorder supervened and after the development of more pronounced splenomegaly, a diagnosis of ALPS was confirmed.",

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T1 - Tubulointerstitial Nephritis in a Patient With Probable Autoimmune Lymphoproliferative Syndrome

AU - Glerup, Mia

AU - Herlin, Troels

AU - Rittig, Søren

AU - Grønbæk, Kirsten

AU - Hokland, Marianne

AU - Hasle, Henrik

PY - 2013/7

Y1 - 2013/7

N2 - Autoimmune lymphoproliferative syndrome (ALPS) is caused by a nonmalignant defective Fas-mediated apoptosis. The main clinical manifestations are chronic lymphadenopathy, splenomegaly, and autoimmune cytopenia. Most patients with ALPS have a FAS germline mutation. ALPS has occasionally been associated with glomerulonephritis and we present the first report of tubulointerstitial nephritis associated with probable ALPS. A 5-year-old girl presented with fever, vomiting, hypertension, and azotemia. No autoantibodies, viral, or streptococcal antibodies were detected. A renal biopsy showed small-vessel vasculitis with normal glomeruli and inflammation in the interstitium. The patient responded to prednisolone treatment and obtained a full renal recovery. Symptoms of connective tissue disorder supervened and after the development of more pronounced splenomegaly, a diagnosis of ALPS was confirmed.

AB - Autoimmune lymphoproliferative syndrome (ALPS) is caused by a nonmalignant defective Fas-mediated apoptosis. The main clinical manifestations are chronic lymphadenopathy, splenomegaly, and autoimmune cytopenia. Most patients with ALPS have a FAS germline mutation. ALPS has occasionally been associated with glomerulonephritis and we present the first report of tubulointerstitial nephritis associated with probable ALPS. A 5-year-old girl presented with fever, vomiting, hypertension, and azotemia. No autoantibodies, viral, or streptococcal antibodies were detected. A renal biopsy showed small-vessel vasculitis with normal glomeruli and inflammation in the interstitium. The patient responded to prednisolone treatment and obtained a full renal recovery. Symptoms of connective tissue disorder supervened and after the development of more pronounced splenomegaly, a diagnosis of ALPS was confirmed.

U2 - 10.1097/mph.0b013e31828ac9fe

DO - 10.1097/mph.0b013e31828ac9fe

M3 - Journal article

C2 - 23588339

SN - 1077-4114

VL - 35

SP - e187-e189

JO - Journal of Pediatric Hematology/Oncology

JF - Journal of Pediatric Hematology/Oncology

IS - 5

ER -

Tubulointerstitial Nephritis in a Patient With Probable Autoimmune Lymphoproliferative Syndrome

Abstract

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