Table 1.

Summary of cases of AA amyloidosis in patients with giant cell arteritis and/or polymyalgia rheumatica.

Patient, Age (yrs), SexGCA or PMR, GCA Biopsy?Amyloidosis Manifestation, Organ InvolvementTherapy for GCA/PMR, Duration of TreatmentTherapy for Amyloidosis, Duration of TreatmentOutcome/Followup
1, 79 F*GCA/PMR, TA biopsy neg, MRI posNephrotic syndrome, renalPrednisone 5–60 mg/day, 1.5 yrsColchicine 0.3 mg/day, 4 moStabilization of kidney function
2, 59 M2GCA/PMR, TA biopsy posRenal, GI, cardiac, lung, thyroid, adrenal, GU, spleenPred 75 mg/day initially, then tapered, 2 yrsPred 75 mg/day initially, then tapered, 2 yrsDeath due to cardiac complications, ESRD
3, 76 F3GCA/PMR, TA biopsy posNephrotic syndrome, renal, GI, cardiac, GU, spleen, adrenalPred 5–60 mg/day, 5 yrsPred 5–60 mg/day, 5 yrsDeath due to ESRD
4, 73 M4GCA/PMR, TA biopsy posDiagnosed at autopsy only: spleen, GI, GU, renal cardiacPred 10–40 mg/day, MTX 15 mg/week, 11 yrsNone (diagnosed at autopsy)Death from complication from VP shunt for NPH
5, 74 F5GCA/PMR, TA biopsy posNephrotic syndrome, renalPred 25 mg/day initially, then refusal to take steroids, unknown durationDeflazacort 60 mg/day, chlorambucil 5 mg/day, unknown durationUnknown
6, 81 F6GCA/PMR, TA biopsy posNephrotic syndrome, renalPred 60 mg/day, then tapered, colchicine 2 mg/day, 18 monthsPred 60 mg/day, then tapered, colchicine 2 mg/day, 18 moImprovement in proteinuria
7, 71 F6GCA/PMR, TA biopsy negNephrotic syndrome, renalPred 5–60 mg/day, 2.5 yrsPred 60 mg/day, then tapered, colchicine 2 mg/day, 6 moStabilization of proteinuria and creatinine
8, 72 M7GCA/PMRNephrotic syndrome, renalNot specified, 16 moPulse pred 300 mg/day × 3, then 70 mg/day, colchicine not tolerated, chlorambucil, 3 yrsESRD
9, 65 M8GCA, TA biopsy, fibrosisNephrotic syndrome, renal, rectalInitially pred 60 mg/day, 1 yrInitially pred 60 mg/day, 1 yrDeath due to ESRD
10, 82 M3PMR, TA biopsy negNephrotic syndrome, renalPred 5–20 mg/day, 4 yrsContinued pred with no dose change, 2 moDeath due to ESRD
11, 66 F9PMRNephrotic syndrome, renalPred 20 mg then tapered over 6 mo, pred and MTX for flare after discharge, 1 yrLow-dose pred to keep inflammatory markers near normalStabilization of creatinine
  • * Our case. GCA: giant cell arteritis; PMR: polymyalgia rheumatica; TA: temporal artery; GI: gastrointestinal; GU: genitourinary; pred: prednisone; ESRD: endstage renal disease; VP: ventriculoperitoneal; NPH: normal pressure hydrocephalus; MTX: methotrexate.