Abstract: Multicentric Reticulohistiocytosis Responding to Tumor Necrosis Factor-a Inhibition in a Renal Transplant Patient

Multicentric Reticulohistiocytosis Responding to Tumor Necrosis Factor-a Inhibition in a Renal Transplant Patient

SEAN E. SHANNON, H. RALPH SCHUMACHER, SALLY SELF, and ALAN N. BROWN

ABSTRACT.

Multicentric reticulohistiocytosis (MRH) is a rare systemic disease that characteristically produces severe erosive arthritis of the distal and proximal interphalangeal joints along with cutaneous nodules over the upper extremities and face, but it can be found in other organs. There is no known etiology, and it often remits spontaneously within 5–10 years, but can result in severe disfigurement. The diagnosis is made when histologic analysis of involved synovium or cutaneous nodules reveals characteristic histopathology. We describe a case of MRH manifesting as erosive arthritis that developed despite immunosuppressive therapy. Based on studies reporting tumor necrosis factor-a (TNF-a) positive cells within MRH lesions, we began treatment with TNF-a inhibition. Within 2 months the patient showed dramatic clinical and serologic improvement that was maintained until she stopped treatment secondary to an upper respiratory tract infection. Once treatment was restarted, the symptoms again improved. (J Rheumatol 2005;32:565-7)

Key Indexing Terms:

HISTIOCYTOSIS
NON-LANGERHANS CELL
IMMUNOSUPPRESSIVE AGENTS
KIDNEY TRANSPLANT
RECEPTORS
TUMOR NECROSIS FACTOR

From the Departments of Rheumatology-Immunology and Pathology, Medical University of South Carolina, Charleston, South Carolina, and the Division of Rheumatology, University of Pennsylvania, Veterans Affairs Medical Center, Philadelphia, Pennsylvania, USA.

S. Shannon, MD, Rheumatology Fellow; A. Brown, Assistant Professor of Medicine; S. Self, MD, Associate Professor of Pathology, Director, Diagnostic Immunology, Medical University of South Carolina; H.R. Schumacher, MD, Professor of Medicine, University of Pennsylvania School of Medicine, Director, Rheumatology-Immunology Center, Philadelphia VAMC.

Address reprint requests to Dr. S.E. Shannon, Medical University of South Carolina, Division of Rheumatology and Immunology, 96 Jonathan Lucas Street, Suite 912, CSB, PO Box 250637, Charleston, SC 29425, USA.

Submitted May 10, 2004; revision accepted October 5, 2004.