Clinical improvement of renal amyloidosis in a patient with systemic-onset juvenile idiopathic arthritis who received tocilizumab treatment: a case report and literature review.

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enalapril 1 endocrinologydiseasesdrugs
hydroxychloroquine 2 endocrinologydiseasesdrugs
methylprednisolone 2 endocrinologydiseasesdrugs
amyloidosis 43 endocrinologydiseases
cyclophosphamide 1 endocrinologydiseasesdrugs

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Select Drug Character Offset Drug Term Instance
cyclophosphamide 15235 TCZ tocilizumab, DMARDs disease-modifying anti-rheumatic drugs, ADA adalimumab, COL colchicine, CYC cyclophosphamide , INF infliximab, ANA anakinra, ABA abatacept, HCQ hydroxychloroquine, LFN leflunomideMany studies have
enalapril 9067 increased from a dose of 8 mg/kg every 4 weeks to a dose of 8 mg/kg every 2 weeks. We also started enalapril at 5 mg/day (0.12 mg/kg/day) for an additional antiproteinuric effect. The other drugs were continued
hydroxychloroquine 5552 the tocilizumab. Therefore, pulse methylprednisolone at 1 g/month, leflunomide at 20 mg/dose, and hydroxychloroquine at 200 mg/day were gradually added to the tocilizumab regimen. The rheumatologist noticed her first
hydroxychloroquine 15302 adalimumab, COL colchicine, CYC cyclophosphamide, INF infliximab, ANA anakinra, ABA abatacept, HCQ hydroxychloroquine , LFN leflunomideMany studies have also demonstrated inadequate efficiency of anti-TNF-α medications
methylprednisolone 3873 of SJIA were quotidian fever, polyarthritis, and pericardial effusion. Although she received pulse methylprednisolone , high-dose prednisolone, and multiple disease-modifying anti-rheumatic drugs from her previous hospital
methylprednisolone 5486 course still waxed and waned. Her arthritis relapsed while receiving the tocilizumab. Therefore, pulse methylprednisolone at 1 g/month, leflunomide at 20 mg/dose, and hydroxychloroquine at 200 mg/day were gradually added
Select Disease Character Offset Disease Term Instance
amyloidosis 51 Title: BMC NephrologyClinical improvement of renal amyloidosis in a patient with systemic-onset juvenile idiopathic arthritis who received tocilizumab treatment: a
amyloidosis 523 arthritis (JIA) is a common rheumatic disease in children and adolescents. Although JIA may cause secondary amyloidosis , this is a rare complication in patients with JIA and other rheumatic diseases. Many previous studies
amyloidosis 964 difficult-to-control SJIA. She developed progressive proteinuria without clinical signs or symptoms of edema. Renal amyloidosis was diagnosed by renal pathologic examination, which demonstrated deposition of eosinophilic amorphous
amyloidosis 1935 patient exhibited a clinical response within 3 months.ConclusionRefractory SJIA associated with renal amyloidosis is an uncommon cause of proteinuria in adolescents. Tocilizumab may be a beneficial treatment for renal
amyloidosis 2051 an uncommon cause of proteinuria in adolescents. Tocilizumab may be a beneficial treatment for renal amyloidosis in patients with SJIA.BackgroundAmyloidosis is a group of systemic or localized diseases characterized
amyloidosis 2288 deposition of amyloid fibrils in multiple organs [[1], [2]]. Organ involvement depends on the subtype of amyloidosis . Secondary or reactive amyloidosis can develop in patients with chronic inflammatory diseases and affects
amyloidosis 2323 multiple organs [[1], [2]]. Organ involvement depends on the subtype of amyloidosis. Secondary or reactive amyloidosis can develop in patients with chronic inflammatory diseases and affects multiple organs such as the kidneys,
amyloidosis 2567 gastrointestinal system, and autonomic nervous system [[3]]. The amyloidogenic precursor protein in secondary amyloidosis is mainly synthesized by the hepatic system in a chronic inflammatory state. Accumulation of amyloid
amyloidosis 2849 Juvenile idiopathic arthritis (JIA) is a common rheumatic disease in children and may cause secondary amyloidosis [[5], [6]]. However, amyloidosis is still a rare complication in patients with JIA and other rheumatic
amyloidosis 2882 is a common rheumatic disease in children and may cause secondary amyloidosis [[5], [6]]. However, amyloidosis is still a rare complication in patients with JIA and other rheumatic diseases [[5], [6]]. Many previous
amyloidosis 3405 heterozygous E148Q mutation in the MEFV gene. Renal pathological examination confirmed the diagnosis of amyloidosis . This case will help general practitioners and pediatricians to recognize the etiology of proteinuria
amyloidosis 6943 results are listed in Table 1.Table 1Clinical characteristics and laboratory resultsParametersBefore amyloidosis treatmentAt 1-year follow-upClinical characteristics - Number of joints involved1911 - CHAQ-DI score1.371.25Immunology -
amyloidosis 8401 showed partial foot process effacement under electron microscopy. These biopsy findings confirmed renal amyloidosis .Fig. 1Depositions of amorphous eosinophilic material (arrow) in the (a) glomerular hilum and (b) arteriolar
amyloidosis 8896 fibrils measuring 8 to 10 nm in diameter (×40,000)Clinical follow-up after renal biopsyBecause the amyloidosis was secondary to uncontrolled SJIA, the tocilizumab was increased from a dose of 8 mg/kg every 4 weeks
amyloidosis 10198 analyzed. A heterozygous c.442G > C (pE148Q) mutation in the MEFV gene was identified.DiscussionRenal amyloidosis is a rare cause of proteinuria in children. The prevalence of amyloidosis is higher in patients with
amyloidosis 10272 identified.DiscussionRenal amyloidosis is a rare cause of proteinuria in children. The prevalence of amyloidosis is higher in patients with JIA than in the general pediatric population [[10], [11]], and among the
amyloidosis 10457 [[10], [11]], and among the seven subtypes of JIA, SJIA is associated with the highest prevalence of amyloidosis [[10], [12]]. In the present case, amyloidosis occurred 10 years after the onset of SJIA, similar to
amyloidosis 10504 JIA, SJIA is associated with the highest prevalence of amyloidosis [[10], [12]]. In the present case, amyloidosis occurred 10 years after the onset of SJIA, similar to a previous study [[12]]. In many types of amyloidosis,
amyloidosis 10613 amyloidosis occurred 10 years after the onset of SJIA, similar to a previous study [[12]]. In many types of amyloidosis , the kidneys are the predominant organ involved. Proteinuria is the most common clinical presentation
amyloidosis 11817 Thai, 24.3% [unpublished data]). Without other polymorphisms in the MEFV gene, the presentation of amyloidosis of our patient may or may not have been related to the heterozygous E148Q mutation. Further functional
amyloidosis 12160 E148Q.Although we cannot classify the specific subtype of the amyloidogenic precursor in our country, AA amyloidosis is the most common subtype in patients with various chronic inflammatory diseases, including SJIA [[3]–[6]].
amyloidosis 12382 Therefore, we treated the patient in this case based on current evidence regarding treatment of AA amyloidosis . The main principle of management is suppression of the inflammatory process and control of the underlying
amyloidosis 12586 the underlying disease [[5], [17]]. The clinical characteristics and treatment of JIA-related renal amyloidosis are shown in Table 2. Three of six patients were diagnosed with SJIA, and two responded to chlorambucil
amyloidosis 12737 patients were diagnosed with SJIA, and two responded to chlorambucil or leflunomide treatment for renal amyloidosis [[18], [19]]. Cantarini et al. [[8]] could not demonstrate the efficacy of colchicine in one patient
amyloidosis 12959 because of loss to follow-up. Some studies have shown the efficacy of chlorambucil for JIA-related amyloidosis [[10], [11]]. One case-cohort study [[10]] revealed that 80% of patients receiving chlorambucil achieved
amyloidosis 13344 controlled trials (RCTs) regarding the efficacy of chlorambucil and leflunomide in patients with JIA-related amyloidosis .Table 2Clinical characteristics and treatment of reported patients with secondary amyloidosis related
amyloidosis 13438 JIA-related amyloidosis.Table 2Clinical characteristics and treatment of reported patients with secondary amyloidosis related to juvenile idiopathic arthritisReferenceAge (y)/SexDuration of JIA before diagnosis of amyloidosis
amyloidosis 13546 amyloidosis related to juvenile idiopathic arthritisReferenceAge (y)/SexDuration of JIA before diagnosis of amyloidosis (y)Systemic features of JIAaESR or CRP, Initial to last FU (mm/h or mg/dL)Organ involvement of amyloidosiseGFR,
amyloidosis 13653 amyloidosis (y)Systemic features of JIAaESR or CRP, Initial to last FU (mm/h or mg/dL)Organ involvement of amyloidosis eGFR, initial to last FU (mL/1.73 m2/min)Proteinuria, Initial to last FU (mg/day)Treatment before diagnosis
amyloidosis 13776 initial to last FU (mL/1.73 m2/min)Proteinuria, Initial to last FU (mg/day)Treatment before diagnosis of amyloidosis Additive treatment after diagnosis of amyloidosisDuration of FU after diagnosis of amyloidosisEfficacy
amyloidosis 13825 Initial to last FU (mg/day)Treatment before diagnosis of amyloidosisAdditive treatment after diagnosis of amyloidosis Duration of FU after diagnosis of amyloidosisEfficacy of amyloidosis treatmentDuarte et al. 2005 [[18]]9/F5.5YesESR
amyloidosis 13870 diagnosis of amyloidosisAdditive treatment after diagnosis of amyloidosisDuration of FU after diagnosis of amyloidosis Efficacy of amyloidosis treatmentDuarte et al. 2005 [[18]]9/F5.5YesESR 125 to 27RenalNAUPCR (mg/mmol)
amyloidosis 13893 amyloidosisAdditive treatment after diagnosis of amyloidosisDuration of FU after diagnosis of amyloidosisEfficacy of amyloidosis treatmentDuarte et al. 2005 [[18]]9/F5.5YesESR 125 to 27RenalNAUPCR (mg/mmol) 1057 to 47MTX, CSA, GCs,
amyloidosis 15636 disease was common [[20]–[23]]. Two studies have shown the efficacy of anti-TNF therapy in treating amyloidosis secondary to autoimmune diseases, including JIA [[24], [25]]. A retrospective study of 15 patients with
amyloidosis 15752 secondary to autoimmune diseases, including JIA [[24], [25]]. A retrospective study of 15 patients with amyloidosis with underlying rheumatic diseases revealed that 54.5% of patients receiving infliximab (81%) or etanercept
amyloidosis 16075 RCTs demonstrated the efficacy of tocilizumab for the treatment of refractory SJIA in patients without amyloidosis [[26], [27]]. Several retrospective studies have demonstrated clinical improvement rates of around 70%
amyloidosis 16236 demonstrated clinical improvement rates of around 70% in adult patients with rheumatic disease with amyloidosis after tocilizumab treatment [[28], [29]]. However, patients with SJIA were not included in these studies;
amyloidosis 16402 patients with SJIA were not included in these studies; thus, the efficacy of tocilizumab treatment for amyloidosis secondary to SJIA remains unclear.The present patient developed secondary amyloidosis despite receiving
amyloidosis 16488 treatment for amyloidosis secondary to SJIA remains unclear.The present patient developed secondary amyloidosis despite receiving tocilizumab, which can be explained by her uncontrolled SJIA. The patient still had
amyloidosis 17296 UPCR had decreased to 0.23 at the 1-year follow-up. Therefore, tocilizumab is beneficial in treating amyloidosis secondary to SJIA when conventional therapies fail. However, the minimum dose of tocilizumab in such
amyloidosis 17489 tocilizumab in such patients is 8 mg/kg every 2 weeks.ConclusionWe have described a case of renal amyloidosis secondary to refractory SJIA in a carrier of the E148Q mutation in the MEFV gene. Physicians should
amyloidosis 17632 a carrier of the E148Q mutation in the MEFV gene. Physicians should be aware of the possibility of amyloidosis development in patients with SJIA, especially when such patients show progressive proteinuria and uncontrolled
amyloidosis 17915 affect clinical outcomes and lead to a better prognosis. Tocilizumab may be beneficial in patients with amyloidosis related to uncontrolled SJIA

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