Background A amyloidosis, is a rare but serious complication of chronic inflammatory conditions including Adult-onset Still’s disease (AOSD). Treatment can be frustrating too.
Objectives We describe the case of refractory AOSD who developed secondary amyloidosis without proteinuria. As she failed to respond to several agents including anti-TNF alpha factory therapy, the treatment with Tocilizumab, an anti –human interleukin-6-receptor inhibitor was introduced. It brought the level of serum amyloid A (SAA) from several hundred times normal to almost normal value after 3 injections
Methods A-27 year old female has carried the diagnosis of AOSD since 2008 after experiencing repeated attacks, fever, malaise, arthropathy, myalgia and pleura-pericarditis. She fulfilled the criteria of Yamaguchi (1) for the diagnosis. Her laboratory workup repeatedly showed a rise in the levels of inflammatory markers including C reactive protein (CRP), ESR, serum ferritin along with leucocytosis during relapses. She failed to respond adequately to several courses of agents including NSAIDs, corticosteroids, methotrexate, leflonamide, anti TNF alpha agents and anti-CD20 (Rituximab). Anti IL-1 was not available locally. Because of the refractory nature of the disease a monthly injection of Tocilizumab (8mg/kg) was commenced with reasonable response evidenced by spacing out relapses and declining of inflammatory markers. Despite the absence of proteinuria during the follow-up period a latent amyloidosis was suspected.
Results The subcutaneous abdominal wall fat for Congo-Red staining was highly positive. Concomitantly, the SAA carried out by Bioscientia, Germany, revealed a level of 1140 mg/L (normal up to 6.4 mg/L). Within the subsequent three injections of tocilizumb the level went down further to 49 mg/L, and then lately to only 14 mg/L.
Conclusions Undoubtedly the decline in the SAA is a reflection of a reduced amyloid activity, which is worth reporting. Tocilizumab, therefore, is a promising agent for treating secondary amyloidosis by suppressing the level of SAA. Such a successful outcome by this agent opens a new frontier for its indications.
Yamaguchi M, Ohta A, Tsunematsu T et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19:424–30
Disclosure of Interest None Declared
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