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Azathioprine as successful maintenance therapy in IgG4-related tubulointerstitial nephritis.

Pozdzik AA, Brochériou I, Demetter P, Matos C, Delhaye M, Devière J, Nortier JL - Clin Kidney J (2012)

Bottom Line: One year later, renal function remains stable.Our clinical observation underlines the importance of biological and radiological long-term follow-up of patients with previous AIP in order to early detect IgG4-related renal involvement.Corticosteroids are the first choice, but in the case of adverse effects or partial remission, AZA could be a useful and safe alternative therapy.

View Article: PubMed Central - PubMed

Affiliation: Nephrology Department, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium ; Experimental Nephrology Unit, Faculty of Medicine, Université Libre de Bruxelles, Brussels, Belgium.

ABSTRACT
A 65-year-old man presented with a progressive increase in plasma creatinine (PCr). Two years before, diffusion-weighted magnetic resonance imaging had revealed a relapse of immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP) associated with sclerosing cholangitis. Bilateral hypointense renal cortical nodules were also described. Kidney biopsy showed patchy disappearance of tubules, sparse interstitial fibrosis and IgG4+ plasma cells (>30 per high power field) leading to the diagnosis of IgG4-related tubulointerstitial nephritis (TIN). Despite methylprednisolone, PCr and serum IgG4 levels remained elevated. Starting azathioprine (AZA) normalized IgG4 levels, which elicited corticosteroid withdrawal after 17 months. One year later, renal function remains stable. Our clinical observation underlines the importance of biological and radiological long-term follow-up of patients with previous AIP in order to early detect IgG4-related renal involvement. Corticosteroids are the first choice, but in the case of adverse effects or partial remission, AZA could be a useful and safe alternative therapy.

No MeSH data available.


Related in: MedlinePlus

Time course of plasma creatinine (open circle) and serum IgG4 levels (closed circle). Grey boxes indicate MPS therapy (started at 1 mg/kg/day, followed by progressively tapered doses every 4 weeks) and the hatched box corresponds to AZA administration (2 mg/kg of body weight/day). Arrow indicates the time of DW-MRI and star indicates the time of kidney biopsy (Time 0).
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fig2: Time course of plasma creatinine (open circle) and serum IgG4 levels (closed circle). Grey boxes indicate MPS therapy (started at 1 mg/kg/day, followed by progressively tapered doses every 4 weeks) and the hatched box corresponds to AZA administration (2 mg/kg of body weight/day). Arrow indicates the time of DW-MRI and star indicates the time of kidney biopsy (Time 0).

Mentions: Partial clinical response was obtained with oral methylprednisolone (MPS) therapy (1 mg/kg/day), as PCr and IgG4 levels remained elevated (Figure 2). The introduction of AZA (2 mg/kg/day) normalized IgG4 levels, eliciting the total withdrawal after 17 months. One year later, PCr is stable (1.8–1.9 mg/dL) and IgG4 levels are within the normal range.


Azathioprine as successful maintenance therapy in IgG4-related tubulointerstitial nephritis.

Pozdzik AA, Brochériou I, Demetter P, Matos C, Delhaye M, Devière J, Nortier JL - Clin Kidney J (2012)

Time course of plasma creatinine (open circle) and serum IgG4 levels (closed circle). Grey boxes indicate MPS therapy (started at 1 mg/kg/day, followed by progressively tapered doses every 4 weeks) and the hatched box corresponds to AZA administration (2 mg/kg of body weight/day). Arrow indicates the time of DW-MRI and star indicates the time of kidney biopsy (Time 0).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400520&req=5

fig2: Time course of plasma creatinine (open circle) and serum IgG4 levels (closed circle). Grey boxes indicate MPS therapy (started at 1 mg/kg/day, followed by progressively tapered doses every 4 weeks) and the hatched box corresponds to AZA administration (2 mg/kg of body weight/day). Arrow indicates the time of DW-MRI and star indicates the time of kidney biopsy (Time 0).
Mentions: Partial clinical response was obtained with oral methylprednisolone (MPS) therapy (1 mg/kg/day), as PCr and IgG4 levels remained elevated (Figure 2). The introduction of AZA (2 mg/kg/day) normalized IgG4 levels, eliciting the total withdrawal after 17 months. One year later, PCr is stable (1.8–1.9 mg/dL) and IgG4 levels are within the normal range.

Bottom Line: One year later, renal function remains stable.Our clinical observation underlines the importance of biological and radiological long-term follow-up of patients with previous AIP in order to early detect IgG4-related renal involvement.Corticosteroids are the first choice, but in the case of adverse effects or partial remission, AZA could be a useful and safe alternative therapy.

View Article: PubMed Central - PubMed

Affiliation: Nephrology Department, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium ; Experimental Nephrology Unit, Faculty of Medicine, Université Libre de Bruxelles, Brussels, Belgium.

ABSTRACT
A 65-year-old man presented with a progressive increase in plasma creatinine (PCr). Two years before, diffusion-weighted magnetic resonance imaging had revealed a relapse of immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP) associated with sclerosing cholangitis. Bilateral hypointense renal cortical nodules were also described. Kidney biopsy showed patchy disappearance of tubules, sparse interstitial fibrosis and IgG4+ plasma cells (>30 per high power field) leading to the diagnosis of IgG4-related tubulointerstitial nephritis (TIN). Despite methylprednisolone, PCr and serum IgG4 levels remained elevated. Starting azathioprine (AZA) normalized IgG4 levels, which elicited corticosteroid withdrawal after 17 months. One year later, renal function remains stable. Our clinical observation underlines the importance of biological and radiological long-term follow-up of patients with previous AIP in order to early detect IgG4-related renal involvement. Corticosteroids are the first choice, but in the case of adverse effects or partial remission, AZA could be a useful and safe alternative therapy.

No MeSH data available.


Related in: MedlinePlus