Idiopathic multicentric Castleman’s disease, infrequent cause of Lupus-like. Case Report.
Main Article Content
Abstract
Castleman’s disease (CD) or angiofollicular lymph node hyperplasia includes a heterogeneous mix of reactive lymphoproliferative processes with well-defined histological features. However, they differ in their localization patterns, clinical expression and etiopathogenesis. There are 4 types, one of them is the multicentric CD that is not associated with any viruses and has recently been called idiopathic MCD (iMCD). iMCD is a lymphoproliferative disorder with specific histopathological characteristics, more than one region of affected lymph nodes and absence of infection associated to human herpesvirus 8 and human immunodeficiency virus (HIV). iMCD covers multiple differential diagnoses and might simulate autoimmune diseases such as systemic lupus erythematosus. The aim of this article is to report the case of a patient with Castleman’s disease and lupus-like presentation. We present the case of a 38-year-old man without morbid history, who presented lumbago, fever, diaphoresis and asthenia with two months of evolution, associated to bilateral cervical adenopathies. General examinations result negative, antinuclear antibodies at a dilution of 1/640 were positive, and extractable nuclear antigens were positive suggesting moderate Systemic Lupus Erythematosus (SLE) plus secondary Sjögren’s. Methylprednisolone and Hydroxycloroquine boli were thus initiated. The patient evolved with anasarca, severe anemia, acidosis, polyserositis and multiple mediastinal adenopathies. Immunoglobulin and cyclophosphamide were thus initiated. He later presented fever, thrombocytopenia and nephrotic syndrome. Biopsy of cervical lymph node reported lymphadenitis with polyclonal plasmacytosis and concentric lymphoid hyperplasia, in agreement with iMCD. Treatment with Rituximab was initiated, which led to the favorable evolution of the patient. iMCD is a systemic inflammatory disease, its presentation corresponds to a constitutional syndrome resulting in a wide differential diagnosis. Every time suspicious adenopathies appear, they must be biopsied since this might lead to a definitive diagnosis.
References
Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 1956;9:822-30.
Fajgenbaum DC. Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Hematology Am Soc Hematol Educ Program. 2018;2018(1):318-325.
Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castleman disease: Novel insights into biology, pathogenesis, and therapy. Blood 2014;123:2924-33.
García AG, Cobo MM, de la Pena JP. Diagnóstico y tratamiento actual de la enfermedad de Castleman. Rev Clin Española 2016;216(3):146-156.
Herrada J, Cabanillas F, Rice L, Manning J, Pugh W. The clinical behavior of localized and multicentric Castleman disease. Ann Intern Med. 1998;128:657-62.
Shariatpanahi S, Pourfarzam S, Gheini M. Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus. Iran J Pathol. 2016;11(3):265-271.
Tokunaga M, Yamada M, Yoshikawa S, Kondo A, Mishima M, Inoue S, Morita T, Tominaga N. Systemic lupus erythematosus with marked eosinophilia and clinical features mimicking TAFRO syndrome. Rinsho Ketsueki. 2018;59(6):688-694.
Wang L, Chen H, Shi J, Tang H, Li H, Zheng W, Zhang F. Castleman disease mimicking systemic lupus erythematosus: A case report. Medicine (Baltimore). 2018;97(38):e12291.
Zhang L, Cao XX, Wang SJ, Zhou DB, Li J. Clinical Features of Patients with Castleman’s Disease Complicated Systemic Lupus Erythematosus. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2016;38(5):543-547.