Patient, Age (yrs), Sex | GCA or PMR, GCA Biopsy? | Amyloidosis Manifestation, Organ Involvement | Therapy for GCA/PMR, Duration of Treatment | Therapy for Amyloidosis, Duration of Treatment | Outcome/Followup |
---|---|---|---|---|---|
1, 79 F* | GCA/PMR, TA biopsy neg, MRI pos | Nephrotic syndrome, renal | Prednisone 5–60 mg/day, 1.5 yrs | Colchicine 0.3 mg/day, 4 mo | Stabilization of kidney function |
2, 59 M2 | GCA/PMR, TA biopsy pos | Renal, GI, cardiac, lung, thyroid, adrenal, GU, spleen | Pred 75 mg/day initially, then tapered, 2 yrs | Pred 75 mg/day initially, then tapered, 2 yrs | Death due to cardiac complications, ESRD |
3, 76 F3 | GCA/PMR, TA biopsy pos | Nephrotic syndrome, renal, GI, cardiac, GU, spleen, adrenal | Pred 5–60 mg/day, 5 yrs | Pred 5–60 mg/day, 5 yrs | Death due to ESRD |
4, 73 M4 | GCA/PMR, TA biopsy pos | Diagnosed at autopsy only: spleen, GI, GU, renal cardiac | Pred 10–40 mg/day, MTX 15 mg/week, 11 yrs | None (diagnosed at autopsy) | Death from complication from VP shunt for NPH |
5, 74 F5 | GCA/PMR, TA biopsy pos | Nephrotic syndrome, renal | Pred 25 mg/day initially, then refusal to take steroids, unknown duration | Deflazacort 60 mg/day, chlorambucil 5 mg/day, unknown duration | Unknown |
6, 81 F6 | GCA/PMR, TA biopsy pos | Nephrotic syndrome, renal | Pred 60 mg/day, then tapered, colchicine 2 mg/day, 18 months | Pred 60 mg/day, then tapered, colchicine 2 mg/day, 18 mo | Improvement in proteinuria |
7, 71 F6 | GCA/PMR, TA biopsy neg | Nephrotic syndrome, renal | Pred 5–60 mg/day, 2.5 yrs | Pred 60 mg/day, then tapered, colchicine 2 mg/day, 6 mo | Stabilization of proteinuria and creatinine |
8, 72 M7 | GCA/PMR | Nephrotic syndrome, renal | Not specified, 16 mo | Pulse pred 300 mg/day × 3, then 70 mg/day, colchicine not tolerated, chlorambucil, 3 yrs | ESRD |
9, 65 M8 | GCA, TA biopsy, fibrosis | Nephrotic syndrome, renal, rectal | Initially pred 60 mg/day, 1 yr | Initially pred 60 mg/day, 1 yr | Death due to ESRD |
10, 82 M3 | PMR, TA biopsy neg | Nephrotic syndrome, renal | Pred 5–20 mg/day, 4 yrs | Continued pred with no dose change, 2 mo | Death due to ESRD |
11, 66 F9 | PMR | Nephrotic syndrome, renal | Pred 20 mg then tapered over 6 mo, pred and MTX for flare after discharge, 1 yr | Low-dose pred to keep inflammatory markers near normal | Stabilization 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.