Abstract
BackgroundIgG4-related disease, described around the years 2000 as a form of autoimmune pancreatitis, is now increasingly accepted as a systemic syndrome. The diagnosis is based on both comprehensive and organ-specific criteria. For the kidney, Mayo clinic classification and the guidelines of the Japanese Nephrology Society are used. Ultimately, together with parameters that characterize every organ or apparatus involved, the key element is the confirmation of growing levels of IgG4 in blood or in tissues.Case presentationWe describe a male patient with chronic renal failure associated to hypertension without proteinuria. IgG4-related disease was diagnosed through renal biopsy. After an initial positive response to steroids, he presented tinnitus, and histological assessment showed cerebral and subsequently cardiac damage, both IgG4-related. This case appears unique for the type of histologically documented cardiac and neurological parenchymal involvement, and at the same time, exemplifies the subtle and pernicious course of the disease. Frequently, blurred and non-specific signs prevail. Here, kidney damage was associated with minimal urinary findings, slowly progressive renal dysfunction and other factors that can be equivocated in the differential diagnosis. Neurological involvement was represented by tinnitus alone, while cardiac alterations were completely asymptomatic.ConclusionsThis report is representative of the neurological and cardiac changes described in the literature for IgG4-related disease, which may be correlated or not with the renal form and highlights the need, in some cases, of targeted therapeutic approaches. In addition to glucocorticoids, as in this case, rituximab may be necessary.
Highlights
IgG4-related disease, described around the years 2000 as a form of autoimmune pancreatitis, is increasingly accepted as a systemic syndrome
This report is representative of the neurological and cardiac changes described in the literature for IgG4-related disease, which may be correlated or not with the renal form and highlights the need, in some cases, of targeted therapeutic approaches
We describe here a particular case report, somehow unique, characterized by both neurological and cardiological involvement, with slightly different features from the currently available literature [6]
Summary
This case report shows how the diagnosis of IgG4-RD is often challenging. In this particular instance, the patient presented a slow deterioration of renal function in concomitance with a series of other factors potentially causing chronic renal damage: high blood pressure, immunological and alloreactive profile. The patient presented a slow deterioration of renal function in concomitance with a series of other factors potentially causing chronic renal damage: high blood pressure, immunological and alloreactive profile Respiratory symptoms, such as hypertrophy of submandibular and paratracheal lymph nodes, allergic rhinitis and/or conjunctivitis, must be considered as an alarm bell. Even if the patient initially responded to steroid therapy, he underwent disease relapse when doses were tapered, and our experience agrees with previous findings indicating that steroids often are not enough to control the disease [24].
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