Abstract
BackgroundMulticentric Castleman’s disease is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6. Although renal complications of Castleman’s disease include AA amyloidosis, thrombotic microangiopathy, and membranoproliferative glomerulonephritis, membranous nephropathy is relatively rare. We experienced a case of secondary membranous nephropathy associated with Castleman’s disease.Case presentationThe patient was a 43-year-old Japanese man who had shown a high zinc sulfate value in turbidity test, polyclonal hypergammaglobulinemia, anemia, and proteinuria. A physical examination revealed diffuse lymphadenopathy, an enlarged spleen and papulae of the body trunk. A skin biopsy of a papule on the patient’s back showed plasma cells in the perivascular area and he was diagnosed with multicentric Castleman’s disease, plasma cell variant. Kidney biopsy showed the appearance of bubbling in the glomerular basement membranes in Periodic acid methenamine silver stain and electron microscopy revealed electron dense deposits within and outside the glomerular basement membranes. Since immunofluorescence study showed predominant granular deposition of IgG1 and IgG2, he was diagnosed with secondary membranous nephropathy associated with Castleman’s disease. He was initially treated with prednisolone alone, however his biochemical abnormalities did not improve. After intravenous tocilizumab (700 mg every 2 weeks) was started, his C-reactive protein elevation, anemia, and polyclonal gammopathy improved. Furthermore, his urinary protein level declined from 1.58 g/gCr to 0.13 g/gCr. The prednisolone dose was gradually tapered, then discontinued. He has been stable without a recurrence of proteinuria for more than 6 months.ConclusionsTocilizumab might be a treatment option for secondary membranous nephropathy associated with Castleman’s disease.
Highlights
Multicentric Castleman’s disease is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6
While unicentric CD (UCD) is thought to be a benign disease because curative resection is possible for most patients [2], multicentric CD (MCD) is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6 (IL-6) [3]
Previous studies have shown that renal complications of Castleman’s disease (CD) mainly include AA amyloidosis, thrombotic microangiopathy (TMA), and membranoproliferative glomerulonephritis (MPGN) [4, 5]
Summary
Castleman’s disease (CD) consists of a lymphoproliferative disorder that was first reported by Castleman et al [1]. He was examined by a local doctor who pointed out hypergammaglobulinemia and anemia. An excisional submental lymph node biopsy showed diffuse interfollicular plasma cell infiltration (Fig. 1a). A skin biopsy of a papule on the patient’s back showed plasma cells in the perivascular area (Fig. 1b). Hematoxylin and eosin (HE) staining showed that there was no inflammatory cell infiltration in the glomeruli (Fig. 1c). The patient’s C-reactive protein (CRP) elevation, anemia, and polyclonal gammopathy improved (Fig. 4). His urinary protein level declined from 1.58 g/gCr to 0.13 g/gCr. The prednisolone dose was gradually tapered, discontinued.
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