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Pt | | | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 |
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Sex | | | M | M | F | F | M | F | F | F | F | F |
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Race | | | C | C | C | C | C | H | C | C | C | C |
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SSc clinical subtype | | | Sine | Limited | Limited | Diffuse | Limited | Limited | Diffuse | Limited | Limited | Sine |
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Disease duration at time of ulcer development (years) | | | NA | 20 | 17 | 4.1 | 4 | 46 | 4 | 26 | 24 | NA |
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Scleroderma-specific antibody | | | U3RNP | Centromere | Centromere | RNA Pol3 | | Centromere | Scl70 | Centromere | Scl70 | Centromere |
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Other Antibodies | | | Nucleolar ANA; RF | Speckled ANA | | Speckled ANA | SSA | Nucleolar ANA | Speckled ANA | Speckled ANA | | |
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Other scleroderma features | | | GI dysmotility, GERD | GI dysmotility, GERD, SICCA, limited skin | GI dysmotility, SICCA, limited skin, joint contractures. | Diffuse skin, SICCA, arthritis, interstitial lung disease, GI dysmotility | SICCA, limited skin, joint contractures | GERD, Telangiectasias | Diffuse skin, interstitial lung disease, GI dysmotility, SICCA, calcinosis | Limited skin, joint contractures, SICCA, GERD, GI dysmotility, calcinosis | Limited skin, arthritis, pulmonary hypertension, GERD, SICCA, GI dysmotility | Calcinosis |
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Ulcer location | | | Left leg | Left medial malleolus | Bilateral malleoli and right posterior ankle | Bilateral malleoli | Bilateral Toes, dorsal foot and heel | Bilateral lateral calf | Right medial malleolus, left dorsal foot | Bilateral malleoli | Left lateral malleolus | Bilateral toes and bottom of feet |
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Venous insufficiency on doppler US | | | − | − | − | − | − | − | − | − | + | − |
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Arterial Disease on ABPI | | | + | + | − | − | − | − | − | − | − | − |
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| | | | | | | | | | | | |
SCREEN 1 | LAC | | + | − | − | + | − | − | + | − | − | − |
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β-2GPI (normal <10 U/mL) | IgG | 39 | 10 | 10 | 10 | 10 | 10 | 41 | 10 | 10 | 10 |
IgA | 10 | 13 | 10 | 10 | 10 | 100 | 23 | 10 | 10 | 10 |
IgM | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 |
|
ACL (normal <10 U/mL) | IgG | 10 | 10 | 10 | 45 | 10 | 10 | 23 | 10 | 10 | 10 |
IgA | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 |
IgM | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 |
|
| | | | | | | | | | | | |
SCREEN 2 | LAC | | + | − | − | + | − | − | + | − | − | NT |
β-2GPI (normal <10 U/mL) | IgG | 23 | 10 | 10 | 19 | 10 | 10 | 10 | 10 | 10 | NT |
IgA | 10 | 20 | 10 | 10 | 10 | 100 | 10 | 10 | 10 | NT |
IgM | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | NT |
ACL (normal <10 U/mL) | IgG | 10 | 10 | 10 | 20 | 10 | 10 | 10 | 10 | 10 | NT |
IgA | 10 | 10 | 10 | 14 | 10 | 10 | 10 | 10 | 10 | NT |
IgM | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | NT |
|
| | | | | | | | | | | | |
Summary APLprofile | | | + | + | − | + | − | + | + | − | − | − |
|
| | | | | | | | | | | | |
Genetic procoagulant profile | MTHFR | | 0 | 1 | 2 | 1 | 1 | 1 | 0 | 1 | 1 | NT |
PAI-1 | | 0 | 1 | 1 | 0 | 2 | 0 | 1 | 0 | 0 | NT |
Prothrombin Gene | | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | NT |
FVL | | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | NT |
|
Summary Genetic procoagulant profile | | | − | + | + | + | − | + | + | + | + | NT |
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Biopsy | | | Fibrin occlusive vasculopathy | No biopsy | Fibrin occlusive vasculopathy | Fibrin occlusive vasculopathy | No biopsy | No biopsy | Fibrin occlusive vasculopathy | Fibrin occlusive vasculopathy | No biopsy | No biopsy |
|
Treatment | | | Enoxaparin 40 mg daily, arterioplasty | Arterioplasty | Enoxaparin 1 mg/Kg twice daily | Enoxaparin stopped due to bleeding Darbepoetin alfa | Pentoxifylline 400 mg three times per day | Enoxaparin 40 mg daily | Enoxaparin 40 mg daily | None | Venous surgery pending | Healed with Nifedipine |
|
Outcome | | | Healed | Healed | Not healed | Healed | Healed | Healed | 50% healing in 3 months | Not healed | Not healed | Healed |
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Total duration of ulcer (months) | | | 36 | 6 | 350 | 10 | 6 | 23 | 36 | 3 | 5 | 6 |
|
Time to healing after initiation of therapy | | | 4 | 6 | — | 3 | 6 | 4 | — | — | — | 3 |
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