CASE REPORTS Secondary (AA-Type) Amyloidosis in Patients With Polymyalgia Rheumatica Almudena Escriba ´ , MD, Enrique Morales, MD, Enriqueta Albizu ´ a, MD, Juan Carlos Herrero, MD, Teresa Ortun ˜ o, MD, Agustin Carren ˜ o, MD, Beatriz Dominguez-Gil, MD, and Manuel Praga, MD ● Several cases of systemic amyloidosis associated with polymyalgia rheumatica (PMR) or giant-cell arteritis (GCA) have been described. Nevertheless, the type of amyloid deposit has not been characterized in most of them. Here we report on two patients with PMR (one with associated GCA) who developed nephrotic syndrome and end-stage renal failure caused by massive amyloid deposition. Immunohistochemical analysis showed that the amyloid deposits were of AA type (secondary amyloidosis) in both cases.  2000 by the National Kidney Foundation, Inc. INDEX WORDS: Secondary amyloidosis; polymyalgia rheumatica (PMR); giant-cell arteritis (GCA). P OLYMYALGIA rheumatica (PMR) is a rela- tively common disorder that affects patients who are more than 50 years of age. 1,2 It is frequently linked to giant-cell (temporal) arteri- tis (GCA), a form of chronic vasculitis that typically involves the cranial branches of the arteries originating from the aortic arch. 3-5 Sev- eral cases of systemic amyloidosis associated with PMR or GCA have been published. 6-11 In this report, we describe two patients with PMR, one of them associated with GCA, who devel- oped nephrotic syndrome and end-stage renal failure caused by massive amyloid deposition. Immunohistochemical analysis showed the sec- ondary type of amyloid deposits (AA protein). CASE REPORTS Case 1 An 82-year-old man was examined in 1994 because of stiffness and pain in the shoulders and hips. He did not report headache, jaw claudication, or visual disturbances. Physical examination showed painful and symmetrical limitations of shoulder and hip movement. Temporal arteries were normal on examination. Laboratory findings at this time included a mild normochromic anemia (hematocrit, 32%; hemoglobin, 10.2 g/dL) with a very high erythrocyte sedimentation rate (106 mm/h). The remaining routine biochemical data were normal, including blood urea nitrogen and serum creatinine levels. Urinary sediment was normal, and proteinuria was negative. Rheumatoid factor, cryoglobulins, and antibodies against antinuclear, anti-DNA, extractable nuclear, and anti- neutrophil cytoplasmic antigens were negative. Serum C3 and C4 fractions of complement were normal. A temporal artery biopsy specimen did not show vasculitis. With the diagnosis of PMR, treatment with oral predni- sone, 15 mg/d, was started. The patient noted a dramatic improvement within a few days. Erythrocyte sedimentation rate decreased to 35 mm/h, and the prednisone dose was gradually reduced. However, several relapses occurred throughout the next 3 years, and the patient was maintained on a prednisone dosage of 5 to 20 mg/d. In November 1997, 4 years after the first bout of PMR, he was admitted to our hospital because of anorexia, weight loss, and mental confusion. Laboratory data showed mild normochromic anemia (hematocrit, 38%; hemoglobin, 11.5 g/dL), with an erythrocyte sedimentation rate of 85 mm/h. Serum creatinine level was 7 mg/dL, and several biochemi- cal data were typical of nephrotic syndrome, such as total protein, 4.5 g/dL; albumin, 2.1 g/dL; cholesterol, 328 mg/ dL; and triglycerides, 305 mg/dL. Nephrotic-range protein- uria (14 g of protein/24 h) was detected. Renal echographies showed normal-sized kidneys, ruling out structural abnor- malities. Serum and urine immunoelectrophoresis did not show monoclonal paraproteins. A percutaneous renal biopsy was performed; massive deposits of an amorphous, eosinophilic material were ob- served in glomeruli, vessels, and tubules. Congo red staining confirmed the amyloid nature of these deposits. Electron microscopy showed the characteristic fibrillar structure of amyloidosis (Fig 1). Routine immunofluorescence showed weak and isolated deposits of immunoglobulin M (IgM) and C3 in some glomeruli. Immunohistochemical studies with antisera to light chains (kappa and lambda) and AA protein showed that the deposits were of AA-type (secondary) amyloid. Renal function continued to worsen, but the patient was not administered chronic dialysis because of his age and From the Nephrology Department, Hospital Universitario 12 de Octubre, Madrid, Spain. Received March 8, 1999; accepted in revised form July 2, 1999. Address reprint requests to Manuel Praga, MD, Nephrol- ogy Department, Hospital 12 de Octubre, Carretera de Andalucı ´a Km 5.400, 28041-Madrid, Spain. E-mail: mpragat@senefro.org  2000 by the National Kidney Foundation, Inc. 0272-6386/00/3501-0020$3.00/0 American Journal of Kidney Diseases, Vol 35, No 1 (January), 2000: pp 137-140 137