Table 2. Case series of autoimmune disease-related CCSVI (n=41).
| Variables | Number | Age (years)/gender | Symptoms and signs | Course of disease | Presumable risk factors | Localization of CCSVI | Abnormal lab test | Treatment |
|---|---|---|---|---|---|---|---|---|
| APS (n=18) | Case 1 | 55/M | Eye discomfort, anxiety and sleep disorder | 7 months | Obesity, hyperlipidemia, hyperuricemia and previous HBV infection, and AT-III deficiency | LIJV-J3 | AT-III, C3, C4↓; anti-β2GP1 Ab↑ (113 RU/mL) | Anti-PLT |
| Case 2 | 75/F | Hearing loss, Head noises, tinnitus, papilledema [1]*, neck discomfort, anxiety and sleep disorder | 30 years | Type 2 DM, HBP, and anemia | RIJV-J3 | IgE↑, C3↓; anti-β2GP1 Ab↑ (47 RU/mL) | None | |
| Case 3 | 29/M | Headache, papilledema [1]*, head noises and sleep disorder | 2 months | Current HBV infection, and anemia | LIJV-J3, RIJV-J1/J3 | C4, C3↓; anti-β2GP1 Ab↑ (40 RU/mL) | Anti-PLT | |
| Case 4 | 30/F | Hearing loss, tinnitus, headache, neck discomfort, papilledema [3]* and IH | 1.5 years | Obesity, hyperhomocysteinemia, and anemia | RTS, RSigS | DD, ESR↑; anti-β2GP1 Ab↑ (50 RU/mL) | Anti-PLT, anti-coagulation, ONSD | |
| Case 5 | 33/M | Headache and papilledema [5]* | 3 months | Obesity and current HBV infection | RTS | Anti-β2GP1 Ab↑ (60 RU/mL) | Anti-PLT, anti-coagulation, stenting, ONSD | |
| Case 6 | 75/F | Dizziness and tinnitus | 2 years | Obesity, hyperlipidemia, HBP, Hashimoto’s thyroiditis, and previous otitis media | Bilateral IJV-J1 | Anti-β2GP1 Ab↑ (65 RU/mL) | Anti-PLT | |
| Case 7 | 62/F | Head noises, neck discomfort, blurry vision and sleep disorder | 2 months | Obesity, type 2 DM, hyperlipidemia, HBP, and CAD | LIJV-J1 | aCL↑ (40 RU/mL) | Anti-PLT | |
| Case 8 | 32/F | Fever, headache, papilledema [3]* and IH | 1 month | Postpartum, anemia | Bilateral TS, SigS, RIJV-J2/J3, LIJV-J3 | WBC, hs-CRP, IL-6, PLT↑; anti-β2GP1 Ab↑ (61 RU/mL) | Corticosteroids | |
| Case 9 | 54/F | Tinnitus, eye discomfort and sleep disorder | 4 years | Obesity, hyperlipidemia, Hashimoto’s thyroiditis, and CAD | RTS, LIJV-J3, RIJV-J3 | ESR↑; anti-β2GP1 Ab↑ (151 RU/mL) | Anti-PLT, anti-coagulation and stenting | |
| Case 10 | 63/F | Headache and head noises | 2 years | HBP | RTS, LIJV-J3 | DD↑; anti-β2GP1 Ab↑ (59 RU/mL) | Anti-PLT, anti-coagulation and stenting | |
| Case 11 | 33/F | Headache, nausea/vomiting, neck discomfort, sleep disorder, and papilledema [4]* | 2 weeks | Hashimoto’s thyroiditis | RTS, RSigS, LIJV-J3 | DD↑; IgG ↑; C4, C3↓; anti-β2GP1 Ab↑ (50 RU/mL) | Anti-coagulation and stenting | |
| Case 12 | 56/F | Tinnitus, hearing loss and sleep disorder | 5 months | HBP, CAD | RIJV-J3 | aCL↑ (42 RU/mL) | Anti-PLT | |
| Case 13 | 19/M | Headache, nausea/vomiting, and eye discomfort | 3 weeks | Obesity | LIJV-J3, RIJV-J3 | AT-III↓; anti-β2GP1 Ab↑ (108 RU/mL) | None | |
| Case 14 | 60/M | Tinnitus, and hearing loss | 1.5 years | None | LIJV-J3 | PS, PC, C3↓; anti-β2GP1 Ab↑ (43.4 RU/mL) | Anti-coagulation and stenting | |
| Case 15 | 69/F | Tinnitus, head noises, eye discomfort and sleep disorder | 15 years | Hyperuricemia, CAD, and Hashimoto’s thyroiditis | LIJV-J2/J3 | DD↑; anti-β2GP1 Ab↑ (135 RU/mL) | Anti-PLT | |
| Case 16 | 39/F | Headache | 20 years | Hashimoto’s thyroiditis | RIJV-J2/J3 | Anti-β2GP1 Ab↑ (53 RU/mL) | None | |
| Case 17 | 62/M | Tinnitus, head noises, neck discomfort, hearing loss, and sleep disorder | 20 years | Hyperuricemia and anemia | LIJV-J3 | PS, PC, C3↓; anti-β2GP1 Ab↑ (81 RU/mL) | Anti-PLT | |
| Case 18 | 61/F | Headache, tinnitus, and sleep disorder | 4 months | Anemia and Hashimoto’s thyroiditis | LTS, LIJV-J3, RIJV-J3 | Fig↑, C3↓, ESR↑; anti-β2GP1 Ab↑ (52 RU/mL) | None | |
| SS (n=8) | Case 19 | 74/M | Headache, head noises, tinnitus, and papilledema [1]* | 3 years | Anemia, Hashimoto’s thyroiditis, and previous ischemic stroke | RIJV-J1 | PS↓; anti-Ro-52 (+++), anti-CENP-B (+++) | Anti-PLT |
| Case 20 | 63/F | Headache, head noises, sleep disorder and papilledema [1]* | 2 years | HBP and Hashimoto’s thyroiditis | LTS, LIJV-J3, RIJV-J3 | C3, C4↓, RF↑; anti-SS-A (+++), anti-Ro-52 (+++) | Anti-coagulation | |
| Case 21 | 61/F | Headache, head noises, tinnitus and hearing loss | 4 months | HBP, CAD, anemia, and previous HBV infection | RTS, RSigS, LIJV-J2/J3, RIJV-J3 | Fig, IgG ↑, PS↓; anti-SS-A (+++), anti-SS-B (+), anti-Ro-52 (+++) | Anti-PLT | |
| Case 22 | 72/M | Head noises, sleep disorder and IH | 3.5 months | Type 2 DM, hyperlipidemia, HBP, CAD, and previous HBV infection | LIJV-J3, RIJV-J3 | PS, C3↓; anti-SS-A (++), anti-Ro-70 (++) | Anti-PLT | |
| Case 23 | 68/M | Tinnitus, hearing loss, and sleep disorder | 10 years | Obesity, hyperhomocysteinemia, previous ischemic stroke, Hashimoto’s thyroiditis | LIJV-J1/J3 | AT-III, PS, PC↓; anti-SS-A, anti-Ro-52, anti-β2GP1 Ab↑ (40 RU/mL) | None | |
| Case 24 | 56/F | Head noises and eye discomfort | 2 years | Hyperlipidemia, HBP, and anemia | RIJV-J3 | IgG, ESR, DD↑; anti-SS-A (+++), anti-SS-B (+), anti-Ro-52 (+++), | Anti-coagulation | |
| Case 25 | 44/F | Headache, papilledema [3]* and IH | 10 years | Obesity and anemia | Bilateral TS, SigS | Anti-SS-A (++), anti-Ro-70 (++) | Optic nerve decompression surgery | |
| Case 26 | 56/F | Eye discomfort, neck discomfort, and sleep disorder | 1 month | Hyperlipidemia, HBP, and previous HBV infection | LSigS | Fig, DD↑, AT-III↑; C3, C4↓, RF, IgG↑; anti-SS-A (++), anti-Ro-52 (++) | Anti-coagulation | |
| IgG4-related disease (n=7) | Case 27 | 53/F | Tinnitus, head noises, hearing loss, sleep disorder and IH | 4 years | HBP and Hashimoto’s thyroiditis | LIJV-J3 | C3, C4↓, RF↑; anti-PNMA2 (CSF) (+) | Anti-PLT |
| Case 28 | 15/F | Headache, nausea/vomiting, and papilledema [2]* | 1.5 months | Obesity, hyperhomocysteinemia, and hyperuricemia | RTS, SSS | Fig↑, PS, PC↓, IgE↑; IgG4 (serum) ↑ (1,760 mg/L) | Anti-coagulation and ONSD | |
| Case 29 | 23/F | Headache, tinnitus, head noises, nausea/vomiting, sleep disorder, papilledema [3]* and IH | 4 months | Hashimoto’s thyroiditis | RTS, RSigS | TBil, DBil, IBILI, ALP↑; IL-6, C3↓; IgG4 (serum)↑ (1,660 mg/L); | Anti-coagulation and ONSD | |
| Case 30 | 78/F | Headache, tinnitus, head noises, hearing loss, sleep disorder, anxiety and IH | 4 years | HBP | LIJV-J3, RIJV-J3 | DD↑, AT-III↓; C3, C4↓; anti-RNP/Sm (+) | Anti-PLT | |
| Case 31 | 25/M | Headache, neck discomfort, sleep disorder, papilledema [2]*, and IH | 2 years | Hyperlipidemia, hyperhomocysteinemia, and previous mastoiditis | SSS, LTS, LSigS, LIJV-J1, RIJV-J2/J3 | IgG4 (CSF) (2,020 mg/L) | Anti-coagulation | |
| Case 32 | 36/F | Tinnitus, papilledema [3], and IH | 1.5 years | None | Bilateral TS, LIJVS-J1 | C3↓; IgG (serum)↑ (1,180 mg/L); IgG4 (serum) ↑ (363 mg/L) | Anti-PLT, anti-coagulation, stenting and ONSD | |
| Case 33 | 32/M | Dizziness, eye discomfort, papilledema [2]*, and IH | 12 years | Chronic nasal sinusitis, Hashimoto’s thyroiditis | Bilateral TS, SigS | ESR↑, IgG4 (serum) (2,550 mg/L) | Anti-PLT, anti-coagulation, stenting | |
| Behcet’s disease (n=2) | Case 34 | 28/M | Headache, head noises, eye discomfort and sleep disorder | 20 years | Hyperhomocysteinemia, and anemia | LIJV | PS, PC, C3, C4↓; IgE↑ | Anti-PLT |
| Case 35 | 23/F | Headache, tinnitus, papilledema [2]* and IH | 3 years | Anemia and Hashimoto’s thyroiditis | Bilateral TS, SigS | PS, C4↓ | Immunomodulatory drugs (corticosteroids+ mycophenolate mofetil) and anticoagulation | |
| Autoimmune hepatitis (n=2) | Case 36 | 74/F | Head noises, tinnitus, eye discomfort, and sleep disorder | 5 years | Hyperlipidemia, chronic nasal sinusitis, Hashimoto’s thyroiditis and previous HBV infection | LIJV-J3 | ALT, AST, LDH, ALP↑; Fig, DD↑, AT-III, PS↓, IgG, IgM, CRP, Hs-CRP, RF, ESR↑; anti-Ro-52(+++), anti-CENP (+++) | Anti-PLT |
| Case 37 | 51/M | Tinnitus and eye discomfort | 2 months | Obesity, hyperlipidemia, HBP, CAD, and previous HBV infection | RIJV-J1 | C3↓; TBil, DBil, IBILI, ALP↑; IgG (CSF), IgG (serum)↑ | Anti-PLT | |
| Wegener’s granulomatosis (n=2) | Case 38 | 67/F | Headache, tinnitus, head noises, hearing loss, eye discomfort, and sleep disorder | 20 years | Obesity, chronic nasal sinusitis, previous mastoiditis, Hashimoto’s thyroiditis, and previous HBV infection | RIJV-J3 | AT-III, C3↓; anti-PR3↑ | Anti-PLT |
| Case 39 | 55/M | Tinnitus, head noises, and neck discomfort | 5 years | Obesity | LIJV-J3, RIJV-J3 | C3, C4↓; anti-PR3 (120 RU/mL) | None | |
| Systemic sclerosis (n=1) | Case 40 | 83/M | Headache | 2 months | Obesity, HBP, type 2 DM, Hashimoto’s thyroiditis | RTS, RSigS, LIJV-J1, RIJV-J2 | Anti-CENP-B (+++) | Anti-PLT and stenting |
| AQP4+ NMOSD (n=1) | Case 41 | 42/M | Progressive blurry vision, vision defect, papilledema [2]*, hyposmia | 1 year | Chronic nasal sinusitis, splenomegaly | LTS, LSigS, LIJV-J2/J3 | WBC (CSF) ↑, AQP4 (serum) (+) | Corticosteroids |
*, the severity of papilledema was evaluated by Frisen scale, presented as papilledema [Frisen scale]. DM, diabetes mellitus; CCSVI, chronic cerebrospinal venous insufficiency; APS, antiphospholipid syndrome; AT-III, anti-thrombin III; RIJV, right internal jugular vein; LIJV, left internal jugular vein; C3, complement 3; C4, complement 4; PLT, platelet; anti-β2GP1 Ab, anti-beta-2 glycoproteins antibodies; HBV, hepatic type B virus; IH, intracranial hypertension; TS, transverse sinus; LTS, left transverse sinus; RTS, right transverse sinus; LSigS, left sigmoid sinus; RSigS, right sigmoid sinus; DD, di-dimer; ESR, erythrocyte sedimentation rate; ONSD, optic nerve sheath decompression; HBP, high blood pressure; DM, diabetes mellitus; CAD, coronary artery disease; WBC, white blood cell; hs-CRP, high-sensitivity C-reactive protein; IL-6, interleukin-6; aCL, anti-cardiolipin antibodies; SigS, sigmoid sinus; PS, protein S; PC, protein C; anti-Ro-52, anti-Ro52 antibodies; anti-Ro-70, anti-Ro70 antibodies; anti-CENP-B, anti-centromere protein B antibodies; Fig, fibrinogen; anti-SS-A, anti-Sjögren’s-syndrome-related antigen A; anti-SS-B, anti-Sjögren’s-syndrome-related antigen B; RF, rheumatic factor; SSS, superior sagittal sinus; CSF, cerebrospinal fluid; TBil, total bilirubin; DBil, direct bilirubin; IBILI, indirect bilirubin; ALP, alkaline phosphatase; anti-PR3, anti-proteinase 3 antibodies; anti-PLT, antiplatelet drugs; AQP, aquaporin-4; AQP4 antibody-positive neuromyelitis optica spectrum disorder.