Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Secondary amyloidosis (AA) is a disorder caused by deposition of insoluble amyloid A fibrils in different tissues and organs. It is a rare and serious complication of rheumatic diseases including spondyloarthropathies (SpA).
The aim of this study was to determine the clinical features, laboratory data, treatment and clinical outcome of patients with SpA who developed AA amyloidosis.
Methods: Design: retrospective (1984–2013). Hospital: academic tertiary hospital. Referral area: 850,000 inhabitants. We reviewed the medical records of 1125 patients with SpA: 509 (45%) psoriatic arthritis (PsA), 263 (23%) ankylosing spondylitis (AS), 128 (11.3%) spondylitis associated with inflammatory bowel disease (IBD), 190 (16.8%) undifferentiated spondyloarthropathies, and 35 (3.1%) reactive arthritis. We selected patients who had a histological diagnosis of AA amyloidosis. Patients with comorbidities that might be associated with AA amyloidosis were excluded.
Results: Fifteen (1.3%) patients with AA amyloidosis were recruited: 11 (73.3%) males and 4 (26.7%) females. Five (33.3%) AS, 5 (33.3%) spondylitis associated with IBD, 4 (26.7%) PsA, and 1 (6.7%) reactive arthritis. Mean age at SpA and AA amyloidosis diagnosis: 35.13 and 57.7 years, respectively. Mean disease duration: 23.9 years. Eleven patients with AA amyloidosis were diagnosed in 1984–2000 and 4 in 2001–2013. Amyloid deposits were observed on biopsy of: rectum (6 cases), kidney (3 cases), subcutaneous fat (4 cases) and bladder (1 case). In one case diagnosis was made incidentally (gallbladder), in the others diagnosis was symptomatic and suspected due to nephrotic syndrome (4), acute renal failure (ARF) (3), non-nephrotic-range proteinuria (3), chronic renal failure (CRF) (2), diarrhoea (1) and macroscopic haematuria (1). The mean values of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) at diagnosis of amyloidosis were 65.4 mm and 25.22 mg/L, respectively. Five patients were treated with infliximab: three showed a clinical improvement, with decreased proteinuria and improved renal function.Ten patients did not receive an anti-TNF treatment, 9 of whom died (2 of CRF, 7 of sepsis). Mortality at one and five years was 30 and 50% respectively. Clinical and analytical parameters are described in Table 1.
|
Age SpA diagnosis (years)/ sex |
22/M |
40/M |
29/F |
59/F |
25/M |
38/M |
28/M |
30/M |
24/M |
40/M |
40/F |
30/M |
44/M |
41/M |
37/F |
|
Disease |
psoriatic arthritis |
psoriatic arthritis |
psoriatic arthritis |
psoriatic arthritis |
ankylosing spondylitis |
ankylosing spondylitis |
ankylosing spondylitis |
ankylosing spondylitis |
ankylosing spondylitis |
spondylitis associated with IBD |
spondylitis associated with IBD |
spondylitis associated with IBD |
spondylitis associated with IBD |
spondylitis associated with IBD |
reactive arthritis |
|
Axial |
yes |
no |
no |
no |
yes |
yes |
yes |
yes |
yes |
yes |
yes |
no |
yes |
yes |
no |
|
Peripheral arthritis |
polyarticular |
oligoarticular |
polyarticular |
polyarticular |
no |
polyarticular |
polyarticular |
polyarticular |
polyarticular |
no |
polyarticular |
polyarticular |
polyarticular |
no |
polyarticular |
|
HLA B 27 |
positive |
ND |
positive |
negative |
ND |
positive |
positive |
negative |
positive |
negative |
negative |
negative |
positive |
negative |
ND |
|
Anti-TNF Therapy/ Indication |
infliximab/ arthritis |
no |
infliximab/ AAa |
no |
no |
no |
infliximab/ AAa |
no |
no |
infliximab/AAa |
no |
no |
infliximab/ AAa |
no |
no |
|
Disease duration (years) |
49 |
28 |
4 |
20 |
37 |
6 |
36 |
39 |
21 |
24 |
20 |
10 |
17 |
35 |
13 |
|
Age at diagnosis of AAa (years) |
71 |
68 |
33 |
56 |
62 |
44 |
64 |
69 |
45 |
67 |
60 |
40 |
61 |
76 |
50 |
|
ESR at diagnosis (mm) |
70 |
27 |
125 |
23 |
ND |
ND |
50 |
80 |
50 |
69 |
82 |
93 |
20 |
96 |
ND |
|
Creatinine at diagnosis (mg/dL) |
1 |
1,2 |
1,1 |
0,9 |
3 |
ND |
2,2 |
ND |
2,5 |
3,2 |
1,8 |
2,3 |
1,7 |
1,8 |
ND |
|
Last creatinine (mg/dL) |
5 |
1,9 |
1 |
1 |
3,3 |
1,8 |
1,8 |
3 |
4 |
6 |
2,1 |
ND |
1,2 |
4,6 |
ND |
|
Biopsy indication |
cholecystectomy |
hematuria |
nephrotic-range proteinuria |
nephrotic-range proteinuria |
ARF |
diarrhea |
chronic renal disease |
proteinuria |
ARF |
AFR |
nephrotic-range proteinuria |
chronic renal disease |
nephrotic-range proteinuria |
proteinuria |
proteinuria |
|
Dialysis |
yes |
yes |
no |
no |
no |
yes |
no |
no |
no |
yes |
no |
yes |
no |
no |
yes |
|
Exitus/ |
yes/sepsis |
yes/sepsis |
no |
no |
yes/sepsis |
yes/sepsis |
no |
yes/renal failure |
yes/renal failure |
no |
yes/sepsis |
yes/sepsis |
no |
yes/sepsis |
yes/sepsis |
M: male; F: female; ND: not determinated; AAa secondary amyloidosis
Conclusion: The frequency of clinically apparent AA amyloidosis in our patients was 1.3%. There was a marked male predominance. Clinical AA amyloidosis was diagnosed at a relatively late stage in SpA. The predominant clinical picture was nephrotic syndrome. The frequency of AA amyloidosis decreases between decades, reflecting better control of the disease with novel therapies. Mortality is high.
Disclosure:
S. Rodriguez-Muguruza,
None;
M. Martínez-Morillo,
None;
S. Holgado,
None;
M. L. Mateo,
None;
J. Sanint,
None;
A. Riveros-Frutos,
None;
J. Cañellas,
None;
X. Tena,
None;
A. Olivé Marqués,
None.
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