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Secondary amyloidosis associated with giant cell arteritis/polymyalgia rheumatica
J Stebbing1, O Buetens, D Hellmann
1Johns Hopkins University Department of Medicine, Baltimore, MD, USA.
Abstract:
Although giant cell arteritis (GCA) is characterized by chronic inflammation, secondary (AA) amyloidosis appears to be an exceptionally rare complication of this disorder. We describe an 84-year-old man with biopsy proven GCA and polymyalgia rheumatica (PMR) who was found at autopsy to have AA amyloid deposition in numerous organs, 9 years after his diagnosis of GCA. Persistent musculoskeletal symptoms, attributed to refractory PMR during the patient's life, were likely due to AA amyloidosis. This unrecognized complication of GCA/PMR confounded his therapy, leading to excessive treatment with corticosteroids and methotrexate. This case shows that the occurrence of AA amyloidosis should be considered in patients with "refractory PMR" developing after a period of treatment, and that autopsies play a vital role in enigmatic cases.
Insights
Giant cell arteritis (GCA) rarely causes secondary amyloidosis (AA). This case highlights how AA amyloidosis can mimic refractory polymyalgia rheumatica (PMR), complicating GCA/PMR treatment.
Area of Science:
- Rheumatology
- Nephrology
- Pathology
Background:
- Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are inflammatory conditions.
- Secondary (AA) amyloidosis is a rare complication of chronic inflammation.
Observation:
- An 84-year-old male with biopsy-proven GCA and PMR was diagnosed with AA amyloidosis post-mortem.
- Amyloid deposition was found in multiple organs nine years after his initial GCA diagnosis.
Findings:
- Persistent musculoskeletal symptoms, initially attributed to refractory PMR, were likely caused by AA amyloidosis.
- The unrecognized AA amyloidosis complicated treatment, leading to excessive corticosteroid and methotrexate use.
Implications:
- AA amyloidosis should be considered in GCA/PMR patients with persistent or refractory symptoms.
- Autopsies are crucial for diagnosing rare complications in complex cases.
- This case underscores the importance of considering systemic complications in chronic inflammatory diseases.