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Updated: Jul 31, 2026

Anti-Nuclear Antibody Screening Using HEp-2 Cells
Published on: June 23, 2014
[Primary systemic amyloidosis presenting as polymyalgia rheumatica and giant cell arteritis]
L Pantoja Zarza1, J Paniagua De La Riva, M Megido Lahera
1Unidad de Reumatología, Hospital del Bierzo, Ponferrada, León.
Abstract:
Primary systemic amyloidosis or AL-amyloidosis is an uncommon disease characterized by the accumulation in vital organs of a fibrillar protein consisting of monoclonal light chains. It is a plasma-cell dyscrasia related to multiple myeloma where clonal plasma cells in the bone marrow produce immunoglobulins that are amyloidogenic. A monoclonal component is present in the serum or urine of 90% of patients. The presentation of most patients with AL amyloidosis is usually related to congestive heart failure, nephrotic syndrome o peripheral neuropathy, but there are unusual features suggesting giant cell arteritis (GCA) and polymyalgia rheumatic (PMR). Although in the majority of AL cases the plasma cells clone is small, the assumption is that the outcome of the disease is uniformly fatal (median survival 12-15 months) and treatment is analogous to those used in malignant proliferative disease. We describe a patient with AL amyloidosis who presented with manifestations of GCA and PMR, and we review the main characteristics of primary amyloidosis.
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