A case of rheumatoid arthritis and limited systemic sclerosis overlap successfully treated with tocilizumab for arthritis and concomitant generalized lymphadenopathy and primary biliary cirrhosis

Abstract

A 57-year-old woman with rheumatoid arthritis (RA) and limited systemic sclerosis (lSSc) was suspected to have lymphadenopathy and primary biliary cirrhosis (PBC). Lymph node biopsy showed reactive follicular lymphadenopathy with intrafollicular plasmacyte infiltration that was interleukin-6 positive by immunohistostaining. Because of gradually worsening arthritis, tocilizumab was administered and arthritis improved markedly. Interestingly, lymphadenopathy and PBC improved simultaneously. This suggested that interleukin-6 might play an important role in reactive lymphadenopathy and PBC associated with RA/lSSc.

Figure 1

Lymphadenopathy at first admission. Lymphadenopathy was seen in both axial lymph nodes at first admission (a). Malignant lymphoma was suspected because lymph nodes echogram showed elliptical shape and reticular or hypoechoic pattern with deviation of lymph node hilum (b).

Figure 2

Histopathological and histoimmunochemical features of lymph node biopsy. Lymph node biopsy showed follicular hyperplasia and germinal center proliferation with narrowed mantle-zone ((a): HE stain, ×20). No dysplasia was seen in germinal center and hemophagocytic cells are scattered in this area ((b): HE stain, ×100). The lymphocytes forming in the germinal center were CD20 positive (c), BCL-2 negative (d), and IL-6 positive (e). Plasmacytes in interfollicular area were also IL-6 positive (f).

Figure 3

Clinical course and changes in articular manifestations, lymph node swelling, and serum ALP, γ-GTP concentration. The improvements of articular manifestations, lymph node swelling, and serum ALP, γ-GTP concentration were closely linked to TCZ therapy.

Figure 4

Change in lymph node size by TCZ therapy. Generalized lymphadenopathy was observed before TCZ therapy (left panel, right inguinal area) but it regressed rapidly with central necrosis after TCZ therapy (right panel, right inguinal area).