Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome – effective treatment with tocilizumab

Abstract

A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented.In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5th year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

К Вопросу О Вторичном Амилоидозе Почек При Ювенильном Ревматоидном Артрите У Детей

“…В биоптатах почек, окрашенных конго красным, в поляризованном свете амилоид изменяет красный цвет окраски на яблочнозеленое свечение, которое обнаруживается в клубочках и мозговом слое почек [40]. Ряд авторов подчеркнули низкую информативность ректальной биопсии для диагностики АА-амилоидоза почек [10,29].…”

“…Имеется несколько сообщений о купировании нефротического синдрома при АА-амилоидозе почек посредством применения иммунобиологических препаратов [10,15,34,36]. Положительный эффект применения рекомбинантного гуманизированного моноклонального антитела к человеческому рецептору ИЛ-6 (тоцилизумаб) описан в трех клинических случаях у детей с АА-амилоидозом почек на фоне ЮРА [10,15,34]. Представлены результаты использования рекомбинантной формы человеческого антагониста рецепторов ИЛ-1 (анакинра) у двоих пациентов с АА-амилоидозом почек, развившимся на фоне ЮРА [36].…”

Renal Lesions of Juvenile Idiopathic Arthritis in Children: A Literature Review

“…In renal biopsy specimens stained with Congo red, in polarized light, amyloid changes the red color staining to apple-green glow, which is found in the glomeruli and the medullary layer of the kidneys [41]. A number of authors emphasized the low informative value of rectal biopsy for AA renal amyloidosis diagnosis [11,30]. To assess the risk of AA-amyloidosis of the kidneys, it is preferable to determine the SAA level in blood.…”

“…The therapeutic insertion tactics of patients with renal AA-amyloidosis are currently actively discussed in the literature. There are several reports on the management of the nephrotic syndrome in AA-amyloidosis of the kidneys with the use of immunobiological drugs [11,16,35,37]. The positive effect of recombinant humanized monoclonal antibody to human IL-6 receptor (tocilizumab) was described in three clinical cases in children with AA-amyloidosis of the kidneys against the background of JIA [11,16,35].…”

“…The structure of renal pathology is represented by secondary amyloidosis, glomerulonephritis, and tubulointerstitial nephritis [10]. The most unfavourable renal lesion in JIA is secondary renal amyloidosis (AA-amyloidosis) [11,12]. To date, the main mechanism of AA-amyloidosis development has been established, which consists in a constant or periodic increase in the concentration of serum amyloid A (SAA) [13].…”