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Published on: March 7, 2019
[Renal involvement by AA amyloidosis in inclusion body myositis]
C Díaz Rodríguez1, A Moreno Fernández, F Sacristán
1Servicio de Nefroloía, F.P Hospital Virxe da Xunqueira, España. candido.diaz.rodriguez@sergas.es
Abstract:
Inclusion body myositis is currently considered a variant of adult inflammatory myopathies. Clinical course is insidious and besides typical proximal muscles disorder, extension to distal ones is found in up to 95% of cases. Mean survival ranges from 3 to 5 years. Infections are the first death cause, secondary to existing disability a few years after diagnosis. Chronic rheumatic diseases related amyloidosis has became the most frequent kind of reactive amyloidosis or AA amyloidosis. Clinical manifestations of AA amyloidosis mainly affect the kidney. We present the case of a woman with Inclusion body myositis and renal involvement by AA amyloidosis. In our review of literature we haven t found any article relating AA amyloidosis with idiopathic inflammatory myopathies of the adult, what would turn this case into the first ever reported. We can probably find the reason in the bad prognosis of this entity. So we propose making a renal biopsy to all those patients with a long lasting rheumatologic disease and unexpected impaired renal function.
Insights
Inclusion body myositis, a rare inflammatory myopathy, can lead to AA amyloidosis affecting the kidneys. This case highlights the importance of renal biopsy in patients with long-standing rheumatologic disease and declining kidney function.
Area of Science:
- Rheumatology
- Nephrology
- Pathology
Background:
- Inclusion body myositis (IBM) is an adult inflammatory myopathy with insidious onset, often affecting both proximal and distal muscles.
- AA amyloidosis, linked to chronic rheumatic diseases, primarily impacts the kidneys.
Observation:
- A case of a woman with Inclusion body myositis and concurrent renal AA amyloidosis is presented.
- Literature review revealed no prior reports linking AA amyloidosis with adult idiopathic inflammatory myopathies.
Findings:
- This represents the first reported case of AA amyloidosis associated with Inclusion body myositis.
- The poor prognosis of IBM may contribute to the rarity of this association.
Implications:
- Renal biopsy is recommended for patients with chronic rheumatologic conditions and unexplained renal impairment.
- Early diagnosis and management of renal involvement in IBM could potentially improve patient outcomes.
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