Background/Purpose: Castleman’s disease is a benign lymphoproliferative disorder characterized histologically by follicular hyperplasia and capillary proliferation with endothelial hyperplasia. In addition, overproduction of Interleukin-6 (IL-6) was shown in the germinal centers of hyperplastic lymph node of patients with multicentric Castleman’s disease (MCD), and is implicated in the pathogenesis of MCD. We evaluated long-term efficacy of IL-6 blockade therapy in a patient with both amyloidosis and interstitial pneumonia secondary to MCD.

Methods: A 61-year-old male had abnormal shadows on his chest radiography pointed out by medical checkup in June 1999. Based on the results of a lymph node biopsy in April 2004, he was diagnosed with plasma cell-type MCD. Abnormal shadow on chest radiography indicating interstitial pneumonia, marked anemia, hypoalbuminemia, hypergammaglobulinemia and proteinuria were observed, and got worse gradually. In November 2007, the patient was admitted to our hospital. He showed marked lymphadenopathy at multiple sites, general malaise, low grade fever, loose stool, edema, coughing and dispnea on exercise were observed. The administration of Tocilizumab (TCZ) (8 mg/kg, every 2 weeks) was initiated in December 2007. As his disease activity was extremely high, it is necessary to use concomitant administration of low dose prednisolone to maintain the inflammation low. We monitored clinical findings, laboratory findings, functional test, imaging, and quality of life (QOL) evaluated by SF36 in the patient for four years after initiation of the IL-6 blockade therapy. Moreover, we examined the efficacy of TCZ for the treatment of amyloidosis by intestinal biopsies both at the baseline and one year after first administration of TCZ.

Results: At the baseline, abnormal laboratory findings were as follows; severe anemia, hypoalbuminemia, hypergammaglobulinemia, an elevated CRP level, an elevated serum amyloid A protein (SAA) level, an elevated IL-6 level and proteinuria (2.39 g/day). The results of the respiratory function test indicated obstructive dysfunction of the lung, and blood gases on room air showed hypoxia. Chest CT scan revealed interstitial pneumonia with multiple cysts distributed throughout the lung fields and generalized lymph node enlargement. Endoscopic biopsy of the stomach and duodenum revealed heavy deposition of AA amyloid at the baseline indicating amyloidosis secondary to MCD. TCZ reduced the levels of both CRP and SAA, and improved anemia and hypoalbuminemia. Disappearance of urinary protein was achieved within a year. Lymph nodes throughout the body decreased in size. The results of the images and functional test demonstrated improvement in the patient’s interstitial pneumonia. One year after initiation of treatment with TCZ, endoscopic biopsy of former sampling site of the stomach and duodenum revealed disappearance of AA amyloid. The result of SF36 indicated the improvement both in physical and mental QOL. No severe side effect were observed during the treatment period.

Conclusion: This case report indicates the long-term efficacy of TCZ for the treatment of MCD. Moreover, TCZ is effective for the treatment of amyloidosis, interstitial pneumonia secondary to MCD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-efficacy-of-tocilizumab-in-a-patient-with-amyloidosis-and-interstitial-pneumonia-secondary-to-multicentric-castlemans-disease-mcd/