Table 1 Summary of previously reported and the present study BD patients with ON
Author, year | Patient No. | Age (years), sex | Duration of the disease (years) | Time between diagnosis of BD and ON, (years) | Symptoms of BD before ON | Major organ involvement prior to ON | Site of ON | Relevant medication before ON | Treatment after diagnosis of ON | Lab test at the time of diagnosis of ON |
|---|---|---|---|---|---|---|---|---|---|---|
Atas et al., 2019 [7] | #1 | 37, Male | 13 | 8 | na | Vascular and NS | Bilateral knee and hip | Azathioprine, Cyclophosphamide | Interferon | aCL IgG, IgM levels: Negative aB2-GPI IgM, IgG levels: Negative LDL:129 (mg/dl) TG:145 (mg/dl) Total Chol:203 (mg/dl) ESR:20 (mm/h) CRP:13 (mg/L) |
#2 | 37, Male | 7 | 2 | na | Vascular | Bilateral knee, left hip, left 3rd MCP | Azathioprine, Cyclophosphamide | Azathioprine | aCL IgG, IgM levels: Negative aB2-GPI IgM, IgG levels: Negative LDL:103 (mg/dl) TG:100 (mg/dl) Total Chol:163 (mg/dl) ESR:3 (mm/h) CRP:10 (mg/L) | |
#3 | 62, Male | 5 | 3 | na | Vascular | Right hip | Azathioprine, Cyclophosphamide | Azathioprine | aCL IgG, IgM levels: Negative aB2-GPI IgM, IgG levels: Negative ESR:48 (mm/h) CRP:16 (mg/L) | |
#4 | 19, Male | 3 | 1 | na | Ocular | Bilateral knee | Azathioprine, cyclosporine, Interferon, Infliximab | Infliximab | aCL IgG, IgM levels: Negative aB2-GPI IgM, IgG levels: Negative ESR:11 (mm/h) CRP:7 (mg/L) | |
#5 | 39, Male | 14 | 10 | na | NS and Ocular | Bilateral knee | Azathioprine, Cyclosporine, Interferon, Infliximab Adalimumab | Adalimumab | aCL IgG, IgM levels: Negative aB2-GPI IgM, IgG levels: Negative LDL:83 (mg/dl) TG:185 (mg/dl) Total Chol: 156 (mg/dl) ESR:48 (mm/h) CRP:100 (mg/L) | |
#6 | 43, Male | 4 | 3 | na | Vascular | Left hip | Azathioprine, Cyclophosphamide | Mycophenolate | aCL IgG, IgM levels: Negative aB2-GPI IgM, IgG levels: Negative ESR:33 (mm/h) CRP:7 (mg/L) | |
#7 | 44, Male | 17 | 16 | na | Ocular | Bilateral hip | Azathioprine, Cyclosporine | Azathioprine | aCL IgG, IgM levels: 12, 11 aB2-GPI IgM, IgG levels: Negative ESR:76 (mm/h) CRP:100 (mg/L) | |
#8 | 33, Male | 14 | 14 | na | Vascular | Bilateral knee | Azathioprine, Cyclophosphamide | Infliximab | aCL IgG, IgM levels: Negative aB2-GPI IgM, IgG levels: Negative LDL:204 (mg/dl) TG:127 (mg/dl) Total Chol:273 (mg/dl) ESR:9 (mm/h) CRP:3 (mg/L) | |
#9 | 25, Male | 4 | 1 | na | Vascular | Bilateral knee | Interferon, Cyclophosphamide | Azathioprine | aCL IgG, IgM levels: 16, 18 aB2-GPI IgM, IgG levels: Negative ESR:32 (mm/h) CRP:57 (mg/L) | |
Banicioiu-covei et al., 2019 [8] | #1 | 36, na | 3 years after the initial symptoms | 1 year after the initial symptoms | erythema nodosum, reduced visual acuity, oral aphthosis | Ocular, Skin | Right hip | Nonsteroidal anti-inflammatory drugs | na | ESR: 96 (mm/h) CRP:18.2 (mg/L) Leukocytes: 17,000/mm3 and 1 week later 24,500/mm3 |
Chang et al., 2001 [10] | #1 | 45, Female | 10 years after initial symptoms | 10 years after initial symptoms | Recurrent oral ulcerations and genital ulcerations, recurrent erythema nodosum, positive pathergy test | Skin | Left knee | - | Colchicine, Aspirin, Nabumetone | Leukocytes: 10,200/mm3 Hematocrit: 39.3% Platelet: 370,000 Total protein: 6.1 (g/dl) Albumin: 3.6 (g/dl) AST: 14 (IU/l) ALT: 26 (IU/l) Creatinine: 0.8 (mg/dl) Cholesterol: 202 (mg/dl) Alkaline phosphatase: 117 (IU/l) ESR: 10 (mm/h) CRP: Negative Urinalysis: Normal Coagulation tests: Normal Rheumatoid factor: Negative Antinuclear antibody: Negative IgG aCL: 18 IgM aCL: Negative Lupus anticoagulant test: Negative HLA-B51: Positive |
#2 | 30, Male | 5 years after the initial symptoms | 2.5 | Recurrent oral ulcerations, recurrent scrotal ulcerations, papulopustular eruptions, erythema nodosum-like lesions, posterior uveitis, seizure and multiple cerebral infarctions, intestinal hemorrhage, and multiple perforations of the cecum and ascending colon, pulmonary infarction | NS, Ocular, GI, and Respiratory system | Right hip | Azathioprine, Cyclosporine | na | WBC: 7100 Hematocrit: 38.9% Platelet: 202,000 Total protein: 6.6 (g/dl) Albumin: 3.9 (g/dl) AST: 12 (IU/l) ALT: 9 (IU/l) Creatinine: 1.0 (mg/dl) Cholesterol: 154 (mg/dl) Alkaline phosphatase: 124 (IU/l) ESR: 7 (mm/h) CRP: < 0.8 (mg/dl) Rheumatoid factor: Negative Antinuclear antibody: Negative Coagulation test: Normal Urinalysis: Normal HLA-B51: Positive IgM aCL antibody: 15 IgG aCL antibody: Negative Lupus anticoagulant: Negative | |
Ersoz et al., 2013 [14] | #1 | 66, Female | 10 | 10 | oral aphthous ulcerations, erythema nodosum, uveitis, episcleritis, positive pathergy test, headaches, hemifacial spasms, and fever | NS | Right hip | Imuran, Colchicine, Amlodipine, and Prednisolone at exacerbations | na | CBC: Normal ESR: Normal CRP: Normal Liver function: Normal |
Essaadouni et al., 2017 [15] | #1 | 26, Female | 6 years after the initial symptoms | 3 | oral ulcers (once a month) and recurring neurological manifestations consisting of headache, seizures and fever, pseudofolliculitis, positive pathergy test | NS | Unilateral hip | Colchicine, Azathioprine, Cyclophosphamide pulse | Tocilizumab | na |
Faezi et al., 2018 [16] | #1 | 26, Female | 9 months after initial symptoms (until shoulder involvement) and 12 months until multifocal ON | 4 months until shoulder involvement and 7 months until multifocal osteonecrosis | Oral aphtosis and decreased visual acuity (pan uveitis and retinal vasculitis) | Ocular | Right shoulder, bilateral knee, bilateral hip, right ankle | Azathioprine, Cyclophosphamide, Infliximab | na | aCL IgG, IgM: Normal Lupus anticoagulant: Normal aB2GP I IgG, IgM: Normal Coagulation tests: Normal Lipid profiles: Normal |
Lin et al., 2008 [17] | #1 | 49, Female | 6 | 6 | recurrent episodes of fever associated with oral and genital ulcers, positive pathergy test, abdominal pain, ileum perforation, diarrhea | GI | Bilateral Hip and left knee | na | Azathioprine | WBC: 13,900 with 68.3% neutrophils, 21.5% lymphocytes, and 9.2% monocytes Hemoglobin: 14.6 (g/dl) Platelet count 159,000 |
Oktayoglu et al., 2012 [11] | #1 | 31, Female | 12 | 12 | Oral ulcerations, genital ulcerations, positive Pathergy test, posterior uveitis, pulmonary thromboembolism and thrombosis in superior vena cava, erythema nodosum like lesions, arthritis | Ocular, Skin, Vascular | Bilateral knee | Colchicine, Cyclophosphamide, Azathioprine, Nonsteroidal anti-inflammatory drugs | Indomethacin | WBC: 5250, Hematocrit: 36.3% Platelet: 383,000 Total protein: 8.3 (g/dL) Albumin: 4 (g/dL) AST: 15 (IU/L) ALT: 14 (IU/L) Creatinine: 0.58 (mg/dL) Alkaline phosphatase: 48 (IU/L) Parathyroid hormone: 36.82 (pg/mL) ESR: 46 (mm/hour) CRP: 0.58 (mg/dL) Thyroid hormone levels: Normal Urinalysis: Normal Coagulation tests: Normal Protein C and Protein S values: Normal aCL IgG and IgM: Negative Rheumatoid factor: Negative Antinuclear antibody: Negative anti-dsDNA: Negative |
Polat et al., 2010 [9] | #1 | 38, Female | 5 years after the initial symptoms | 7 months | Recurrent oral ulcers with 5-year duration and genital ulcers persisting for 1 year, acute pan uveitis (left eye), chronic uveitis (right eye), positive pathergy test | Ocular | Bilateral knee | Azathioprine, Colchicine, Warfarin, Acetazolamide | na | CRP: 68 (mg/dl) ESR: 60 (mm/h) The other hematological and biochemical tests are normal aCL IgM and IgG antibodies: Negative |
Vaiopoulos et al., 2019 [18] | Aseptic bone necrosis was seen in 3 male patients (left hip, right hip, and both hips) | |||||||||
Varoglu et al., 2017 [13] | #1 | 36, Male | 1 | 1 | Oral aphthous ulcerations, gait disorder, diplopia, and speech disorder, genital aphthous ulcerations | NS | Bilateral hip | - | na | HLA B51: Positive The markers of collagen tissue disease: Negative Hematological markers (fibrinogen, antithrombin, and protein C and S): Normal Factor V Leiden, prothrombin G20210A gene mutations, and MTHFR enzyme: Negative The plasma Vit B (12), folic acid, and homocysteine levels: Normal |
Yapar et al., 2001 [12] | #1 | 35, Male | na | na | Uveitis | Ocular | Bilateral knee | Cyclosporine A, Nonsteroidal anti-inflammatory drugs | na | Na |
The present study | #1 | 37, Male | 4 | 15 months | Oral aphthosis, Genital aphthosis, pseudofolliculitis, pan ophthalmitis, GI aphthosis, CNS vasculitis | Ocular, Skin, NS, GI | Left Hip | Azathioprine, Cyclophosphamide, Alendronate | na | Pathergy test: Negative HLA B51: Positive ESR: High Hyperlipidemia: Negative |
#2 | 25, Male | 4 | 2 | Oral aphthosis, Erythema Nodosum, pan ophthalmitis, DVT, oligoarthritis | Skin, Ocular, Vascular, Musculoskeletal | 2 Hips 2 Shoulders | Azathioprine, Methotrexate, Cyclophosphamide, Infliximab | na | Pathergy test: Negative ESR: High HLA B51: Negative Hyperlipidemia: Positive Fatty liver: Negative Osteoporosis: Positive | |
#3 | 28, Male | 9 | 1 | Oral aphthosis, Genital aphthosis, Erythema Nodosum, aneurysm, monoarthritis, Epididymitis, | Skin, Vascular, Musculoskeletal | Left Hip | Azathioprine, Cyclophosphamide, Anticoagulant | na | Pathergy test: Negative HLA B51: Negative ESR: High Hyperlipidemia: Negative Fatty liver: Negative Cushingoid: Negative | |
#4 | 28, Male | 6 | 7 months | Oral aphthosis, Genital aphthosis, Skin Aphthosis, pan ophthalmitis, monoarthritis | Skin, Ocular, Musculoskeletal | Right Hip | Azathioprine, Cyclophosphamide, Alendronate | na | Pathergy test: Positive HLA B51: Negative ESR: High Antiphospholipid antibodies: Negative Hyperlipidemia: Negative Fatty liver: Negative Cushingoid: Negative | |
#5 | 19, Male | 10 | 6 months | Oral aphthosis, Genital aphthosis, pan uveitis, DVT | Ocular, Vascular | Bilateral hip | Methotrexate, Alendronate | na | Pathergy test: Positive HLA B51: Negative ESR: less than 10mm/h Antiphospholipid antibodies: Negative Hyperlipidemia: Negative Fatty liver: Negative Cushingoid: Negative | |
#6 | 36, Male | 12 | 8 | Uveitis, Oral aphthosis, pseudo folliculitis, pan ophthalmitis | Ocular, Skin | Right Hip | Azathioprine, Methotrexate, Alendronate | na | Pathergy test: Negative HLA B51: Positive ESR: High Antiphospholipid antibodies: Negative Hyperlipidemia: Positive | |
#7 | 38, Male | 12 | 8 | Oral aphthosis, Genital aphthosis, pan uveitis, arthralgia | Ocular, Musculoskeletal | Bilateral hip | Methotrexate, Alendronate | na | Pathergy test: Positive HLA B51: Positive ESR: less than 10mm/h Antiphospholipid antibodies: Negative Hyperlipidemia: Negative | |
#8 | 17, Female | 5 | 1 | Oral aphthosis, pan ophthalmitis, oligoarthritis | Ocular, Musculoskeletal | Bilateral hip | Azathioprine, Cyclophosphamide, Alendronate | na | Pathergy test: Positive HLA B51: Negative ESR: less than 10mm/h Antiphospholipid antibodies: Negative Hyperlipidemia: Negative Osteoporosis: Negative | |
#9 | 17, Male | 7 | 19 months | Oral aphthosis, Pseudo Folliculitis, aneurysm, CNS vasculitis | Skin, Vascular, NS | Bilateral hip | Methotrexate, Cyclophosphamide, Alendronate, Aspirin | na | Pathergy test: Positive HLA B51: Negative ESR: less than 10mm/h Antiphospholipid antibodies: Negative Hyperlipidemia: Negative Osteoporosis: Positive Cushingoid: Positive | |
#10 | 31, Male | 8 | 20 months | Oral aphthosis, Genital aphthosis, Pseudo Folliculitis, pan ophthalmitis | Skin, Ocular | Bilateral hip | Methotrexate, Alendronate | na | Pathergy test: Positive HLA B51: Negative ESR: less than 10mm/h Hyperlipidemia: Negative Osteoporosis: Positive | |
#11 | 36, Male | 8 | 1 | Oral aphthosis, pan ophthalmitis, arthralgia, Epididymitis, CNS vasculitis | Ocular, Musculoskeletal, NS | Right Hip | Azathioprine, Cyclophosphamide, Alendronate | na | Pathergy test: Negative HLA B51: Negative ESR: High Hyperlipidemia: Negative Fatty liver: Negative Osteoporosis: Positive | |
#12 | 24, Male | 6 | 40 months | Oral aphthosis, Pseudo folliculitis& Erythema Nodosum, pan uveitis, CNS vasculitis | Skin, Ocular, NS | Bilateral hip | Methotrexate, Cyclophosphamide | na | Pathergy test: Positive HLA B51: Positive ESR: High Antiphospholipid antibodies: Negative Hyperlipidemia: Negative Fatty liver: Negative Osteoporosis: Positive Cushingoid: Negative | |
#13 | 20, Male | 6 | 8 months | Oral aphthosis, Genital aphthosis, Erythema Nodosum, pan ophthalmitis, pulmonary Aneurysm, | Skin, Ocular, Vascular | Bilateral hip | Azathioprine, Anticoagulant | na | Pathergy test: Negative HLA B51: Negative ESR: less than 10mm/h Antiphospholipid antibodies: Negative Hyperlipidemia: Negative Fatty liver: Negative Osteoporosis: Positive Cushingoid: Negative | |
#14 | 39.5 Male | 15 | 9.5 | Oral aphthosis, Pseudo folliculitis, pan ophthalmitis, | Skin, Ocular | Bilateral hip | Methotrexate | na | Pathergy test: Negative HLA B51: Positive ESR: less than 10mm/h Antiphospholipid antibodies: Negative Hyperlipidemia: Negative Fatty liver: Negative Osteoporosis: Positive Cushingoid: Positive | |