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Real pain in the neck: giant cell arteritis presenting with non‐necrotising fasciitis and fever

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creatinine stabilised at 132 μmol/L. Monoclonal gammopathy of undetermined significance (MGUS) is defined as the presence of a circulating paraprotein Click to show full abstract

creatinine stabilised at 132 μmol/L. Monoclonal gammopathy of undetermined significance (MGUS) is defined as the presence of a circulating paraprotein <30 g/L, the absence of lytic bone lesions, anaemia, hypercalcaemia and renal failure related to the paraprotein, and bone marrow infiltration of plasma cells of less than 10%. Although MGUS does not typically cause renal impairment, an entity termed monoclonal gammopathy of renal significance (MGRS) has recently been used to describe kidney disease that is caused by a pathogenic monoclonal immunoglobulin without meeting the criteria for myeloma. Treatment of MGRS differs from that of MGUS, and requires chemotherapeutic agents usually reserved for multiple myeloma. Recurrence of glomerulonephritis post-transplantation in patients with a circulating monoclonal protein has been reported, and often leads to rapid graft loss. Despite a low burden of paraprotein, this patient’s recurrent glomerulonephritis post-transplantation was thought to be consistent with MGRS. Aggressive targeted therapy of the underlying paraprotein with bortezomib and plasma exchange successfully maintained reasonable graft function and achieved resolution of the histopathological changes of MPGN post-transplantation. Only one similar case has been reported prior to this, which highlights the need for increased recognition of this condition and the importance of early and aggressive targeted treatment.

Keywords: giant cell; neck giant; cell arteritis; pain neck; real pain; post transplantation

Journal Title: Internal Medicine Journal
Year Published: 2019

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