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IgG4-related membranous glomerulonephritis and generalized lymphadenopathy
without pancreatitis: a case report
#MMPMID28446143
Huart J
; Grosch S
; Bovy C
; Moutschen M
; Krzesinski JM
BMC Nephrol
2017[Apr]; 18
(1
): 139
PMID28446143
show ga
BACKGROUND: IgG4-related disease is a recently described pathologic entity. This
is the case of a patient with nephrotic syndrome and lymphadenopathy due to
IgG4-related disease. Such a kidney involvement is quite peculiar and has only
been described a few times recently. Renal biopsy showed a glomerular involvement
with membranous glomerulonephritis in association with a tubulo-interstitial
nephropathy. Moreover, the patient was not suffering from pancreatitis. CASE
PRESENTATION: The patient is a middle-aged man of Moroccan origin. He has
developed recurrent episodes of diffuse lymphadenopathies, renal failure and
nephrotic syndrome. Renal biopsies showed membranous glomerulonephritis.
DISCUSSION AND CONCLUSION: The diagnostic approach of this atypical presentation
is discussed in this case report as well as diagnostic criteria, therapeutic
strategies, biomarkers and pathophysiology of IgG4-related disease. IgG4-related
membranous glomerulonephritis is a well-established cause of membranous
glomerulonephritis. It must be sought after in every patient with a previous
diagnosis of IgG4-related disease and in every patient with this histological
finding on renal biopsy. Corticoids are still the first-line treatment of
IgG4-related disease. New therapeutic strategies are needed to avoid
glucocorticoids long term side-effects. Interestingly, the patient was prescribed
cyclophosphamide in addition to glucocorticoids for an immune thrombocytopenia.
This treatment had a very good impact on his IgG4-related disease.
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