F. Gohlke et al., AUTOIMMUNE FINDINGS RESEMBLING CONNECTIVE-TISSUE DISEASE IN A PATIENTWITH CASTLEMANS-DISEASE, Clinical rheumatology, 16(1), 1997, pp. 87-92
Multicentric angiofollicular lymphnode hyperplasia (multicentric Castl
eman's disease) may be associated with acute phase reaction and severa
l autoimmune features. Since lymphadenopathy is a common feature in co
nnective tissue disease, a clear distinction between the different dis
ease entities may be difficult. We describe a 26-year-old male patient
with predominant cervical lymphadenopathy, hepatosplenomegaly and pol
yserositis, diagnosed as collagen disease. He showed several autoimmun
e features including autoimmune haemolytic anaemia, cryoglobulinaemia,
positive antinuclear and anti smooth muscle antibodies, serum immune
complexes and a sensorimotor polyneuropathy. Under immunosuppressive t
herapy with prednisolone and azathioprine, only partial remission was
achieved, Repeated lymph node biopsy together with the clinical featur
es led to the diagnosis of multicentric Castleman's disease in this pa
tient nine years later. Interleukin-6 (IL-6) seems to play an importan
t role in the pathogenesis of clinical and serum biochemical features
in patients with Castleman's disease.