Table 1.
patient | WD diagnosis | Musculoskeletal manifestation | Laboratory findings | Extra-articular manifestations | |||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
GI-tract | neurology | cor | skin | ||||||||||||||||||
# | sex | age at first clinical manifestation (years) | time to diagnosis of WD (months) Follow-up after diagnosis (months) |
Articular manifestation/number of involved joints/pattern of joint involvement | Radiographic findings | ACR/EULAR Criteria DAS28 (ESR) |
CRP (mg/L) ESR (mm) |
hemoglobin (♀ 11.6–15.5 g/dl) (♂ 13.5–17.6 g/dl) |
SFA (cells/μl) | abdominal pain | weight loss | loss of appetite | nausea | depression | dysgeusia | polyneuropathy | headache | pericarditis | hyperpigmentation | erythema | Immuno-suppressive treatment (treatment duration in months) |
1 | m | 38 | 180 60 |
Symmetric polyarthritis (12 large, 40 small): wrists, MCP, PIP, atlanto-axial joint, hips, knees |
Erosive arthritis | 7 6.28 |
84.5 22/45 |
7.3 | 500 | X | X | X | X | X | X | Pred (132), SSZ (4), MTX (43), LEF (26), ETN (3), ADA (4), ABA (8), RTX (6), TCZ (3) | |||||
2 | m | 64 | 47 48 |
Symmetric polyarthritis (10 large, 2 small): wrists, elbows, hips, knees, ankles |
Erosive arthritis | 7 3.58 |
8.2 26/50 |
12.8 | 1100 | X | Pred (35), MTX (5), LEF (1), ADA (4), TCZ (3) | ||||||||||
3 | m | 45 | 56 36 |
Asymmetric polyarthritis (1 large, 5 small): MCP joints and left knee | Non erosive arthritis | 5 4.46 |
4.9 27/57 |
11.9 | 7200 | X | - | ||||||||||
4 | m | 34 | 124 56 |
Asymmetric polyarthritis (3 large, 3 small): MCP, PIP, left wrist, right knee | Non erosive arthritis | 5 4.34 |
18.3 24/54 |
14.7 | 7800 | X | Pred (6), MTX (4) | ||||||||||
5 | m | 57 | 129 57 |
Symmetric arthritis (3 large, 2 small): shoulders, wrists, and left hip | Osteo-proliferation on PIP and MTP, no erosions | 5 4.1 |
37.2 53/89 |
12.5 | No data | X | Pred (4), MTX (8), LEF (7) | ||||||||||
6 | m | 55 | 1 30 |
Symmetric arthralgias (2 large, 3 small): MCP, PIP, elbows, vertebral column, sterno-costal joints, axial manifestation | Osteopenia of the hands, no erosions | 4 2.5 |
12 22/45 |
15.1 | No data | Pred (2) | |||||||||||
7 | f | 50 | 42 24 |
Asymmetric poly-arthritis (6 large, 4 small): shoulders, wrists, MCPs, knees, and MTPs | Erosive arthritis | 5 4.5 |
106 44/78 |
10.9 | 1300 | X | X | X | X | X | Pred (33), MTX (27), LEF (8), ABA (12) |
Abbreviations: ABA abatacept, ADA adalimumab, CRP C-reactive proteine, DAS28 disease activity score 28, ESR erythrocyte sedimentation rate, ETN etanercept, GI-tract gastrointestinal tract, Hb hemoglobine, LEF leflunomide, MCP metacarpophalangeal joints, MTP metatarsophalangeal joints, MTX methotrexate, pred prednisone, PIP proximal interphalangeal joints, WD whipple’s disease, RTX rituximab, SFA synovial fluid analysis, SSZ sulfasalacine, TCZ tocilizumab