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Gender/age, race | Time- to diabetes diagnosis | Time-to resistance | ANA titer/pattern | Acanthosis nigricans | SLE characteristics | ACRI-AAI | Maximum daily insulin dose | Treatment | Final outcome |
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1. F, 62 years, African American. Present case | 3 months | 3 months (catabolic symptoms) | 1 : 2560 speckled | Yes | Raynaud’s phenomenon Lymphopenia Hypocomplementemia | AAI | 2400 U daily dose | Pulse steroid + prednisolone | Remission (metabolic control) |
2. F, 47 years, Asian. [4] | 3 months | 3 months (catabolic symptoms) | Positive speckled | Yes | Fever Oral ulcers Weight loss Axillar lymphadenopathies Leukopenia | ACRI | 12,000 U daily dose | Pulse steroid + 20 mg for 4 weeks | 2 years remission |
3. F, 60 years, African American. [8] | 1 year (hypoglycemia) | 3 months (catabolic symptoms) | Not reported | Yes | Not reported | ACRI | Hypoglycemias with no antidiabetic drugs (despite glucocorticoids, glucagon and octreotide) | 3 immunoglobulin cycles + prednisolone 60 mg daily + azathioprine 100 mg daily | Persistant hypoglycemias (12 months) |
4. F, 8 years, Latina. [9] | Unknown | 3 months (hypoglycemia) | 1 : 640 | Yes | Nephritis Dermatomyositis Raynaud’s phenomenon | ACRI | Hypoglycemias with doses of 1.1 U/kg/day | Mycophenolate + prednisolone + immunoglobulin + rituximab + plasmapheresis + Bortezomib | Persistant hypoglycemias (12 months) |
5. F, 39 years, African American. [10] | 6 months (catabolic symptoms) | 1 month (hypoglycemia) | 1 : 2560 speckled | Yes | Antiphospholipid antibodies | AAI | 1500 U daily | Rituximab + pulse high dose dexamethasone each 4 months for 4 days + maintenance with azathioprine | Remission (12 months follow-up) |
6. M, 63 years, Asian. [11] | 4 months (catabolic symptoms) | 4 months (uncontrolled hyperglycemia) | 1 : 320 | Yes | Nephritis Cryoglobulinemia | ACRI | 306 U daily | Methylprednisolone 40 mg/day + cyclophosphamide | Remission (unknown follow-up) |
7. M, 60 years, Asian. [12] | Unknown | Unknown | 1 : 2560 speckled | No | Ataxia photosensitivity Thrombocytopenia Lymphopenia Hypocomplementemia | ACRI | Continuous infusion 9 mU/kg/min | Rituximab + prednisolone + cyclosporine | Remission (unknown follow-up)—nephrotic syndrome development |
8. M, 44 years, Asian. [13] | 1 year (hyperglycemia) | Unknown (hypoglycemia) | Positive—unknown titers | Yes | Polyarthralgias Hypocomplementemia Hypergammaglobulinemia | ACRI | Hypoglycemias with no antidiabetic drugs | Rituximab | Remission (unknown follow-up) |
9. F, 46 years, Asian. [14] | No diagnosis | 3 months | Positive—unknown titers | No | Raynaud’s phenomenon Thrombocytopenia Hypocomplementemia Nephritis | Negative | 600 U daily | Immunoglobulin + cyclophosphamide + leflunomide | Remission (unknown follow-up) |
10. F, 38 years, Asian[15] | Unknown | Unknown (hypoglycemias) | Positive—unknown titers | No | Photosensitivity Interstitial lung disease | ACRI | Hypoglycemias with no antidiabetic drugs | Prednisolone 30 mg/day + chloroquine 300 mg/day + azathioprine 50 mg/day | Remission (unknown follow-up) |
11. F, 38 years, Caucasian. [16] | Unknown | 2 months (renal failure and catabolic symptoms) | Positive 1 : 1280 speckled | Yes | Nephritis Malar rash Myalgias Arthralgias | ACRI | 2400 U daily | Mycophenolate + pulse steroids + plasmapheresis + rituximab + IV immunoglobulin | Persistant hypoglycemia with glucose supplement requirement and continuous enteral nutrition |
12. F, 50 years, African American. [17] | Unknown | Unknown | Positive | No | Unknown | ACRI | 1300 U daily | 1 rituximab cycle + 3 pulse steroid | Remission (16 months follow-up) |
13. M, 62 years, African American. [17] | Unknown | Unknown | Positive | No | Antiphospholipid antibodies | ACRI | 1250 U daily | 2 rituximab cycles + 3 pulse steroid | Remission (12 months follow-up) |
14. M, 64 years, Caucasian. [17] | Unknown | Unknown | Positive—unknown titers | No | Unknown | ACRI | 1800 U daily | 2 rituximab cycles + 5 pulse steroid | Remission (3 months follow-up) |
15. M, 59 years, Asian. [18] | No diagnosis | 1 month (catabolic symptoms) | Positive 1 : 80 speckled | No | Raynaud’s phenomenon arthritis Alveolar hemorrhage Lymphadenopathies Pancytopenia with AIHA Hypocomplementemia | ACRI | 1800 U daily | 3 Cyclophosphamide cycles + prednisolone + Cyclosporine + Metformin | Remission (15 months follow-up) |
16. M, 37 years, Asian. [19] | Unknown | 3 months (hypoglycemias) | Positive—unknown titers | No | Oral ulcers Fever Arthralgias Photosensitivity Skin lesions | ACRI AAI | Hypoglycemias with no antidiabetic drugs | Prednisolone 60 mg/day + chloroquine 250 mg/day + cyclophosphamide 600 mg every 2 weeks for 6 months | Remission (unknown follow-up) |
17. F, 50 years, from India. [20] | No diagnosis | Unknown (catabolic symptoms) | Positive 1 : 100 homogeneous | Yes | Oral ulcers Weight loss Fatigue Anemia arthritis Skin lesions | ACRI | 2000 U daily | Methylprednisolone pulse + prednisolone + Azathioprine | Remission (unknown follow-up) |
18. F, 23 years, Asian. [21] | No diagnosis | Unknown (catabolic symptoms) | Positive 1 : 320 speckled | Yes | Nephritis Hypocomplementemia | ACRI | Unknown | Methylprednisolone pulse + prednisolone | Initial remission Death due to P. jirovecii pneumonia |
19. F, 13 years, African American. [22] | No diagnosis | 3 months | Unknown | No | Unknown | ACRI | 450 U daily rosiglitazone metformin | Prednisolone 60 mg daily | Unknown |
20. F, 40 years, African American. [23] | No diagnosis | 1 month (asymptomatic hyperglycemia) | Unknown | Yes | Nephritis | ACRI | 4500 U daily | Methylprednisolone pulse + cyclophosphamide | Remission (unknown follow-up) |
21. F, 37 years, African American. [24] | No diagnosis | 6 months | Positive—unknown titers | Yes | Arthritis Raynaud’s phenomenon | ACRI | 3000 U daily | Methylprednisolone pulse + Prednisolone + 5 plasmapheresis cycles + 6 cyclophosphamide cycles | Remission (11 months follow-up) |
22. F, 27 years, African American. [25] | No diagnosis | Unknown | 1 : 640 | Yes | Pericarditis Arthritis | ACRI | 1200 U daily | 2 cyclophosphamide cycles + prednisolone + maintenance with mycophenolate mofetil | Remission (unknown follow-up) |
23. F, 50 years, Asian. [26] | Unknown | 1 month | 1 : 2560 homogeneous | Yes | Hypocomplementemia | ACRI | 610 U daily | Prednisolone 30 mg + IFG-1 | Remission (unknown follow-up) |
24. F, 16 years, Latin. [27] | Unknown | 2 months (catabolic symptoms) | 1 : 1280 | Yes | Nephritis Pancytopenia Serositis | ACRI | Unknown | Methylprednisolone pulse + 6 cyclophosphamide cycles | Remission (24 months follow-up) |
25. M, 69 years, Asian. [28] | No diagnosis | 13 months (hypo and hyperglycemia) | 1 : 1280 speckled | Yes | Raynaud’s phenomenon Hypocomplemententemia Nephritis | ACRI | Unknown | 2 methylprednisolone pulses + Cyclophosphamide + prednisolone 30 mg daily | Remission (unknown follow-up) |
26. F, 59 years, Caucasian. [29] | No diagnosis | 15 months (catabolic symptoms and hypoglycemias) | Positive speckled (no titers) | No | Hypocomplementemia Leukopenia | ACRI | Unknown | Prednisolone for 3 months (80–40 mg/day) | Remission (unknown follow-up) |
27. F, 24 years, Caucasian. [30] | No diagnosis | 9 months (hypoglycemias) | Positive 1 : 64 | No | Discoid lupus Lymphadenopathies Fever Serositis Arthritis leukopenia Lymphopenia Hypocomplementemia | ACRI | Unknown | Prednisolone for 3 months (20–5 mg/day) | Remission (unknown follow-up) |
28. F, 52 years, African American. [31] | No diagnosis | 1 month (hypoglycemias) | Positive 1 : 2084 homogeneous | No | Arthritis Alopecia Hypocomplementemia Leukopenia | ACRI | Hypoglycemias with no antidiabetic drugs despite of daily 300 gr glucose intravenous infusion | Prednisolone (120–10 mg/day) | Remission (unknown follow-up) |
29. F, 49 years, African American. [32] | 2 years | 14 months (catabolic symptoms) | Positive 1 : 160 speckled | Yes | Alopecia Leukopenia Nephritis | ACRI | 24,000 daily | prednisone | Death (unknown cause) |
30. F, 40 years, African American. [32] | No diagnosis | Unknown (catabolic symptoms) | Positive 1 : 360 speckled | Yes | Arthralgias Leukopenia Nephritis Neurolupus | ACRI | 6000 UI daily | Pulse steroids | Death (unknown cause) |
31. F, 23 years, African American. [32] | Unknown | 9 months (catabolic symptoms) | Positive—unknown titers | Yes | Arthritis Leukopenia Hypocomplementemia Nephritis | ACRI | 2600 U daily | 3 plasmapheresis and lymphapheresis cycles + 3 cyclophosphamide cycles + Prednisone | Remission (unknown follow-up) |
32. F, 51 years, African American. [33] | 4 years | 8 months (catabolic symptoms) | Positive 1 : 160 speckled | Yes | Alopecia Hypocomplementemia | No tested | 25,000 U daily | No drugs | Spontaneous remission (20 months follow-up) |
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