Clinical Rheumatology

, Volume 16, Issue 1, pp 87–92 | Cite as

Autoimmune findings resembling connective tissue disease in a patient with castleman's disease

  • F. Gohlke
  • E. Märker-Hermann
  • S. Kanzler
  • M. Mitze
  • K. -H. Meyer Zum Büschenfelde
Case Report


Multicentric angiofollicular lymphnode hyperplasia (multicentric Castleman's disease) may be associated with acute phase reaction and several autoimmune features. Since lymphadenopathy is a common feature in connective tissue disease, a clear distinction between the different disease entities may be difficult. We describe a 26-year-old male patient with predominant cervical lymphadenopathy, hepatosplenomegaly and polyserositis, diagnosed as collagen disease. He showed several autoimmune features including autoimmune haemolytic anaemia, cryoglobulinaemia, positive antinuclear and anti smooth muscle antibodies, serum immune complexes and a sensorimotor polyneuropathy. Under immunosuppressive therapy with prednisolone and azathioprine, only partial remission was achieved. Repeated lymph node biopsy together with the clinical features led to the diagnosis of multicentric Castleman's disease in this patient nine years later. Interleukin-6 seems to play an important role in the pathogenesis of clinical and serum biochemical features in patients with Castleman's disease.

Key words

Multicentric Castleman's Disease Autoimmune Features Collagen Disease Interleukin-6 POEMS-Syndrome 


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  1. 1.
    Castleman B, Iverson L, Menendez VP. Localized mediastinal lymph-node hyperplasia resembling thymoma. Cancer 1956; 9: 822–30.Google Scholar
  2. 2.
    Leger-Ravet MB, Peuchmaur M, Devergne O, Audouin J, Raphael M, Van Damme J et al. Interleukin-6 gene expression in Castleman's disease. Blood 1991; 178: 2923–30.Google Scholar
  3. 3.
    Schur PH. Clinical features of SLE. WN Kelly, ED Harris, S Ruddy, CB Sledge, editors. In: Textbook of Rheumatology, 4th ed. Philadelphia: W.B. Saunders Company 1993: 1017–42.Google Scholar
  4. 4.
    Frizzera G. Castleman's disease and related disorders. Semin Diag Pathol 1988;5:346–64.Google Scholar
  5. 5.
    Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972; 29: 670–83.Google Scholar
  6. 6.
    Diebold J, Tulliez M, Bernadou A, Audouin J, Tricot G, Reynes M et al. Angiofollicular and plasmacytic polyadenopathy: a pseudotumourous syndrome with dysimmunity. J Clin Pathol 1980; 33: 1068–76.Google Scholar
  7. 7.
    Yoshizaki K, Kuritani T, Kishimoto T. Interleukin-6 in autoimmune disorders. Semin Immunol 1992; 4: 155–66.Google Scholar
  8. 8.
    Frizzera G, Peterson BA, Bayrd ED, Goldman A. A systemic lymphoproliferative disorder with morphologic features of Castleman's disease: Clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985; 3: 1202–16.Google Scholar
  9. 9.
    Petersen BA, Frizzera G. Multicentric Castleman's disease. Semin-Oncol 1993; 20: 634–47.Google Scholar
  10. 10.
    Yoshizaki K, Matsuda T, Nishimoto N, Kuritani T, Taeho L, Aozasa K, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman's disease. Blood 1989; 74: 1360–67.Google Scholar
  11. 11.
    Beck JT, Hsu SM, Wijdenes J, Bataille R, Klein B, Vesole D, et al. Jagannath, S. Brief report: Alleviation of systemic manifestations of Castleman's disease by monoclonal anti-interleuin-6 antibody. N Eng J Med 1994; 330: 602–5.Google Scholar
  12. 12.
    Brandt SJ, Bodline DM, Dunbar MB, Dunbar CE, Nienhuis AW. Dysregulated interleukin 6 expression produces a syndrome resembling Castleman's disease in mice. J Clin Invest 1990; 86: 592–99.Google Scholar
  13. 13.
    Tang B, Matsuda T, Akira S, Nagata N, Ikehara S, Hirano T et al. Age-associated increase in interleukin-6 in MRL/lpr mice. Int Immunol 1991; 3: 273–8.Google Scholar
  14. 14.
    Krishnan J, Danon AD, Frizzera G. Reactive lymphadenopathies and atypical lymphoproliferative disorders. Am J Clin Pathol 1993; 99: 385–96.Google Scholar
  15. 15.
    Bradwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnik DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: The POEMS syndrome. Medicine 1980; 59: 311–22.Google Scholar
  16. 16.
    Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E, Tsubaki T, et al. The Crow-Fukase syndrome: A study of 102 cases in Japan. Neurology 1984; 34: 712–20.Google Scholar
  17. 17.
    Takatsuki K, Yodoi J, Uchiyama T, Sagawa K. Plasma cell dyscrasia with polyneuropathy and endocrine disorder: Review of 36 patients. Neurol Med 1977; 7: 483–93.Google Scholar
  18. 18.
    Mandler RN, Kerrigan DP, Smart J, Kuis W, Villiger P, Lotz M. Castleman's disease in POEMS a syndrome with elevated interleukin-6. Cancer 1992; 69: 2697–2703.Google Scholar
  19. 19.
    Adelman HM, Cacciatore ML, Pascual JF, Mike JM, Alberts WM, Wallach PM. Case report: Castleman's disease in association with POEMS. Am J Med Sci 1994; 307: 112–14.Google Scholar
  20. 20.
    Miralles GD, O'Fallon JR, Talley NJ. Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N Engl J Med 1992; 327: 1919–23.Google Scholar
  21. 21.
    Hosaka S, Kondo H. Three cases of Castleman's disease mimicking the features of collagen disease. Ryumachi 1994; 34: 42–47.Google Scholar
  22. 22.
    Nanki T, Tomiyama J, Arai S. Mixed connective tissue disease associated with multicentric Castleman's disease. Scand J Rheumatol 1994; 23: 215–17.Google Scholar

Copyright information

© Clinical Rheumatology 1997

Authors and Affiliations

  • F. Gohlke
    • 2
  • E. Märker-Hermann
    • 2
  • S. Kanzler
    • 2
  • M. Mitze
    • 1
  • K. -H. Meyer Zum Büschenfelde
    • 2
  1. 1.Department of PathologyJohannes Gutenberg-UniversityMainzGermany
  2. 2.I. Medizinsche Klinik und PoliklinikJohannes Gutenberg-Universität MainzMainzGermany

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