Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome - effective treatment with...

Area of Science:

• Pediatric Rheumatology
• Nephrology
• Immunology

Background:

• Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disease in children.
• Severe JIA can present with systemic complications, including secondary amyloidosis.
• Secondary amyloidosis can lead to nephrotic syndrome, significantly worsening patient prognosis.

Purpose of the Study:

• To report a case of generalized onset JIA complicated by secondary amyloidosis and nephrotic syndrome.
• To describe the challenges in managing a severe JIA case with multiple comorbidities.
• To evaluate the efficacy of tocilizumab in achieving remission in a refractory JIA patient with amyloidosis.

Main Methods:

• Case report detailing the clinical course of a pediatric patient with severe JIA.
• Diagnosis of amyloidosis confirmed via rectal biopsy.
• Review of sequential immunosuppressive therapies including prednisolone, cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide.
• Assessment of treatment response to tocilizumab based on clinical and laboratory parameters.

Main Results:

• The patient presented with severe JIA and developed nephrotic syndrome due to secondary type A amyloidosis.
• Previous treatments failed to achieve sustained remission and were complicated by frequent infections.
• Tocilizumab therapy resulted in clinical and laboratory remission.
• After 42 months of tocilizumab, the patient showed no joint pain or edema and no signs of nephrotic syndrome.

Conclusions:

• Tocilizumab is an effective treatment option for severe juvenile idiopathic arthritis complicated by secondary amyloidosis and nephrotic syndrome.
• Early diagnosis and aggressive management are crucial for patients with severe JIA and systemic complications.
• This case highlights the potential of targeted biologic therapy in achieving long-term remission in refractory pediatric autoimmune diseases.