Abstract
IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.
Highlights
IgG4-Related Disease (IgG4-RD) is a rare fibroinflammatory disorder that can affect almost any organ, characterized by lymphoplasmocytoid infiltrate, obliterative phlebitis, and storiform fibrosis often associated with eosinophilia and increased levels of IgG4 [1]
We report 4 cases of IgG4-RD with renal involvement that exemplify the different pattern of renal disease that can be observed in this disorder, outlining the clinical course and therapeutic approach
The case we describe in this report is of particular relevance, because renal histology shows a glomerulonephritis compatible with Associated Vasculitis (AAV), with rupture of Bowman capsulae and fibrinoid necrosis, together with a tubulointerstitial involvement and IgG4+plasmacells, more typical of IgG4-RD
Summary
IgG4-RD is a rare fibroinflammatory disorder that can affect almost any organ, characterized by lymphoplasmocytoid infiltrate, obliterative phlebitis, and storiform fibrosis often associated with eosinophilia and increased levels of IgG4 [1]. Rituximab is an anti CD20 monoclonal treatment that demonstrates a dramatic efficacy in IgG4-RD, thanks to depletion of B cells and reduction of inflammatory infiltrate: treatment with Rituximab in the early stages of the disease can reverse fibrosis Even in this case, clinical remission may last 6–18 months. A 47 years old woman was evaluated in our outpatient clinics for AIP diagnosed 3 years before in another hospital (abdominal pain, hyperamilasemia; “Salt and pepper,” non-homogeneous pancreatic parenchyma at echography; serum IgG4 256 mg/dL) She had been treated with budesonide 9 mg/die and was under treatment with anti-IL-5 (Mepolizumab) for severe asthma. A renal biopsy was performed, showing mild thickening of glomerular basal membrane and focal mesangial expansion; minimal interstitial fibrosis and IgG4-negative lymphomonocytic infiltrate; minimal tubular atrophy (5% of tubuli) in the absence of vascular abnormalities. After surgical drainage of abdominal abscess, the patient’s conditions improved and no relapse of IgG4-RD in any localization was observed in follow up
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