23 November 2025
: Case report 
Idiopathic Intracranial Hypertension as the Initial Manifestation of Systemic Lupus Erythematosus: A Case Report and Literature Review
Challenging differential diagnosis, Diagnostic / therapeutic accidents, Management of emergency care, Rare coexistence of disease or pathology
Abdulaziz Almalki AE 1, Asayel Abdullah Alosaimi BEF 2, Abdulhameed Sarriyah
AEFG 2*, Sarah Alqasem AEF 2, Qusai Alwazna AEF 3, Faisal Theeb Alqahtani AEF 2
DOI: 10.12659/AJCR.950115
Am J Case Rep 2025; 26:e950115
Table 1 Summary of published papers of idiopathic intracranial hypertension (IIH) as the initial presenting manifestation of systemic lupus erythematosus (SLE).
| Author and Year | Type of study | Sex | Initial presentation | Serology | Associated with lupus nephritis, or Central Nervous System (CNS) involvement in lupus |
|---|---|---|---|---|---|
| Sudhakar et al (2024) []15 | Case report | F | Chronic holocranial headache and occasional episodes of projectile vomiting for the previous 6 months and then developed blurring of vision for the previous month | Not reported | No |
| Rajasekharan et al (2013) []6 | Case report | F | Headache with episodes of transient blurred vision; intermittent fever; reddish, scaly lesions on both cheeks | Antinuclear antibody, anti-double-stranded (ds) DNA registered positively in high titres | No |
| Maloney (2013) []12 | Case report | F | Headache, vomiting, and arthralgias | Positive direct Coombs test; positive antinuclear antibodies (ANA); positive anti-ds DNA (anti-dsDNA); false-positive Venereal Disease Research Laboratory (VDRL) test; reduced complement levels. Anti-Smith (Sm) antibodies, anticardiolipin antibodies, and lupus anticoagulant were negative | No |
| Bettman et al (1968) []11 | Case report | F | Migratory arthralgia, then anorexia | ANA, anti-dsDNA registered positively in high titres | No |
| Carlow & Glaser (1974) []16 | Case report | F | Refractory anemia, fever, and general malaise | ANA, anti-dsDNA positive | No |
| DelGiudice et al (1986) []17 | Case series | F | Not reported | One had normal serology, 2 had positive serology | No |
| Katsuyama et al (2015) []10 | Case report | F | High fever with bilateral abducens nerve palsy | High titer of anti-dsDNA antibodies and hypocomplementemia | No |
| Pugliese et al (2024) []18 | Case report | F | Chronic severe headache and was found to have papilledema. She reported several systemic symptoms including hair loss, skin dryness, and edema | Hypocomplementemia; positive ANA; positive anti-Ro (SSA), anti-Sm, anti-ribonucleoprotein (RNP), and anti-dsDNA antibodies by enzyme immunoassay | Yes, Class V lupus nephritis |
| Kim et al (2012) []7 | Retrospective cohort study | All female (8/8) | Mostly Intractable headache (duration 8–65 days), dizziness, diplopia, blurred vision, and nausea with vomiting | ANA: 8/8 positive; anti-dsDNA: 8/8 positive; anti-Ro: 6/8 positive; anti-ribosomal P: 2/8 positive; hypocomplementemia: low C3 (8/8), low C4 (4/8) | Yes, lupus nephritis: 5/8; CNS lupus (seizure/psychosis): 4/8 |
| Barahona-Hernando et al (2009) []19 | Case Report | F | Severe headache, blurred vision, diplopia | Positive ANA, positive anti-dsDNA, low complement levels | Yes |
| Li & Chan (1990) []20 | Case series | All female 3/3 | Neurological symptoms | Not reported | No |
| Chaves-Carballo et al (1999) []21 | Case report with literature review | F | Severe headache (1-month duration), progressive vomiting (1 week), left abducens palsy (5 days) | ANA, anti-dsDNA positive, with hypocomplementemia | Yes (type II mesangial proliferation) |
| Dave et al (2008) []3 | Retrospective | F | Headache with pulse synchronous tinnitus and blurred vision | Positive ANA, lupus nephritis (Class VI) | Yes (Class VI), depression, anxiety |
| Mathew et al (2012) []22 | Case report | M | Fever, headache, vomiting (6 months), growth retardation, absent secondary sexual characteristics (Tanner stage 1) | ANA positive; anti-dsDNA positive; anti-ribosomal P protein: strongly positive (+++); hypocomplementemia | Yes (Class IV-S) |
| Salvant et al (2019) []8 | Case report | F | One-year history of headaches, progressing to severe episodes with vomiting, photophobia, and vision changes | ANA and anti-dsDNA positive | No |
| Vachvanichsanong et al (1992) []23 | Case report | M | Edema, headache, nephritis for 1 month | Positive ANA (1: 640), positive anti-dsDNA (1: 160), and low C3 (0.21 g/L) | Yes |
| Donald et al (1973) []24 | Case series | F (4) | Headache, nausea, and papilledema | Positive LE preparations, positive antinuclear factor | Yes |
| Green et al (1995) []25 | Case series | F (3) | Headache, papilledema, and neurological deficits | Positive ANA, anti-DNA antibodies, low complement levels | Yes |
| Padeh & Passwell (1996) []26 | Case report | F | Headache, dizziness, nausea, decreased visual acuity, and diplopia | Elevated titers of anti-DNA antibodies and positive results for anti-Sm and anti-RNP antibodies were found | No |
| Yoo et al (2001) []27 | Case report | F | Intractable headache, dizziness, fatigue, nausea, and vomiting | Positive ANA (1: 320, speckled pattern), anti-dsDNA, anti-Ro antibodies | Yes |
| Komura et al (2002) []28 | Case report | F | Fever, headache, nausea, diplopia, and arthralgia | Positive ANA, positive anti-dsDNA, low complement levels | Yes |
| Sbeiti et al (2003) []29 | Case report | F | Severe headache, blurred vision, and diplopia | Positive ANA, positive anti-dsDNA, low complement levels | Yes |
| Kuyucu et al (2007) []30 | Case report | F | Two-month history of headache, fever, and malaise | ANA (1: 40, speckled), anti-dsDNA (44 IU/mL) | No |
| Hershko et al (2008) []5 | Retrospective cohort study | 9 Female, 1 male | 3/10 patients had IIH as their first SLE symptom | 10/10 patients were ANA positive; 7/10 anti-dsDNA positive | Yes, 6/10 had lupus nephritis, 5/10 had additional CNS symptoms (seizures, psychosis, migraine, chorea) |
| Kalanie et al (2019) []31 | Case report | F | One-month history of progressively deteriorating bilateral frontotemporal headache of throbbing quality | Positive ANA, positive anti-dsDNA, low complement levels (C3 and C4), positive lupus anticoagulant | Yes |
| Ang et al (2018) []32 | Case report | F | Two-week history of headache, cognitive impairment (MMSE: 16/30), emotional lability, joint pain, oral ulcers, photosensitivity, and alopecia | Positive ANA (high titer 1: 2560, speckled pattern), hypocomplementemia, high erythrocyte sedimentation rate, negative anti-dsDNA | Yes (IIH) |
| Omer et al (2022) []33 | Case report | M | Severe bilateral headaches, nausea, decreased visual acuity, recurrent epistaxis, and macroscopic hematuria | Positive ANA at 176.85 U (>60 U is positive) and a borderline anti-dsDNA antibody 77.7 IU/mL; positive anti-Sm antibodies; hypocomlemetemia; positive anti-RNP | Yes |
| Tse et al (2012) []34 | Case report | F | Bilateral frontal headaches, blurred vision, fatigue, and weight gain | Positive ANA (1: 1280), positive anti-dsDNA, low complement levels (C3: 0.32 g/L, C4: 0.05 g/L) | Yes |
| Georgakopoulos et al 2011) []35 | Case report | F | Severe headache, blurred vision, and nausea | Positive ANA, positive anti-dsDNA, low complement levels (C3 and C4), positive lupus anticoagulant | Yes |
| Cocco et al (2023) []36 | Original research | SLE: female: male=63: 6; neuropsychiatric SLE (NPSLE): female: male=33: 3 | Mood abnormalities (27.7%); seizures (16.7%); cognitive dysfunction (16.7%); psychosis (11.1%); and demyelinating syndrome (8.3%) | ANA: 100% positive in both groups; anti-dsDNA: 49.3% SLE, 63.9% NPSLE; anti-Sm: 13.0% SLE, 30.6% NPSLE; anti-ribosomal P: 7.2% SLE, 30.6% NPSLE; and low C3/C4: more frequent in NPSLE | No |
| Shah et al (2018) []37 | Case report | F | Severe headache, blurred vision, and fatigue | Positive ANA, positive anti-dsDNA, low complement levels (C3 and C4), positive lupus anticoagulant | Yes |
| Xue et al (2009) []14 | Case report and literature review | F | Intractable headache and extremity swelling | ANA: 1: 10 000 (granular); anti-dsDNA: negative; anti-Ro/SSA and anti-RNP: positive | No |
| Laassila et al (2022) []38 | Case report | F | Multiple episodes of headache over 2 weeks, worsening in intensity, resistant to analgesics, followed by horizontal diplopia | Positive antinuclear antibodies (titer: 1/640) | Yes |
| Silberberg & Laties (1973) []24 | Case report | F | Severe headache, blurred vision, and diplopia | Positive ANA, positive anti-DNA antibodies, and low complement levels | No |
| Shust et al (2024) []39 | Case report | F | Headache, numbness in hands, and worsening memory | Not reported | Yes |
| Srimanan et al (2022) []40 | Case report | F | High-grade fever, malaise, loss of appetite, headache, and lost eyesight | Positive ANA, positive anti-dsDNA, low complement levels, positive anti-beta 2 glycoprotein 1 IgG/IgM/IgA | Yes |
| Our case | Case report | F | Headache and blurred vision for 1 week, associated with generalized fatiguability and bilateral lower limb swelling for 1 month | Positive ANA, positive anti-DNA antibodies, low complement level | Yes |
| Sudhakar et al (2024) []15 | Yes | Corticosteroids | Complete resolution of IIH | ||
| Rajasekharan et al (2013) []6 | Yes | Treated with intravenous mannitol, oral glycerine, and methylprednisolone 1 g once daily (o.d.), which was started on the fourth day and was continued for 3 days followed by cyclophosphamide 25 mg daily. | Fever and headache subsided by the third day of initiating corticosteroids, and she was fully symptom-free by 1 week. Repeat fundus examination after 5 days showed that papilloedema decreased. She was discharged with cyclophosphamide tablets with an advice to review after 25 days | ||
| Maloney (2013) []12 | Yes | Pulse methylprednisone 1 g was subsequently administered for 3 days followed by prednisone 1 mg/kg/day. | Patient initially improved with methylprednisolone and was discharged on prednisone and hydroxychloroquine but defaulted follow-up and treatment, leading to a relapse of IIH. One year later, hospitalization revealed lupus nephritis (class IV) with subnephrotic proteinuria, although serum creatinine stayed normal | ||
| Bettman et al (1968) []11 | Yes | Steroids | Symptom and papilledema was improved and resolved | ||
| Carlow & Glaser (1974) []16 | No | Systemic steroids | Symptoms resolved | ||
| DelGiudice et al (1986) []17 | No 0/3 | One had steroid bolus, 3 had systemic steroids | Resolution (3) | ||
| Katsuyama et al (2015) []10 | Yes | Methylprednisolone pulse therapy | Remission was subsequently maintained with prednisolone at a dose of 3 mg/day for 5 years | ||
| Pugliese et al (2024) []18 | Yes | Methylprednisolone 1 g pulses for 3 days, followed by prednisone 50 mg daily (after prior deworming); mycophenolate mofetil 2 g daily; chloroquine 150–250 mg; acetazolamide; losartan; levothyroxine; calcium and vitamin D supplementation | Adequate response and symptoms control. The patient did not experience any adverse effects from the medication | ||
| Kim et al (2012) []7 | Yes 3/8 | High-dose i.v. steroids (methylprednisolone/dexamethasone): 8/8; i.v. cyclophosphamide pulse: 2/8; with acetazolamide: 3/8; and mannitol: 7/8 | All patients resolved headaches without complications. Follow-up cerebral spinal fluid pressure: normalized in 4/4 retested patients | ||
| Barahona-Hernando et al (2009) []19 | Yes | High-dose corticosteroids (methylprednisolone), acetazolamide | Improved with resolution of symptoms and normalization of intracranial pressure | ||
| Li & Chan (1990) []20 | No 0/3 | 3/3 Steroids | Resolution (3) | ||
| Chaves-Carballo et al (1999) []21 | Yes | Methylprednisolone i.v. (1000 mg/day for 3 days), oral prednisone (20 mg/day), naproxen, hydrochloroquine | Rapid improvement after steroids; stable at 1-year follow-up | ||
| Dave et al (2008) []3 | Yes | Acetazolamide, prednisone (tapered slowly) | Improved with medical therapy | ||
| Mathew et al (2012) []22 | Yes | Pulse methylprednisolone (25 mg/kg/day for 5 days), i.v. cyclophosphamide (750 mg monthly for 6 months). | Fever and headache subsided; papilledema disappeared. Erythrocyte sedimentation rate and anti-dsDNA normalized. | ||
| Salvant et al (2019) []8 | Yes | Started on acetazolamide, received high-dose glucocorticoids, started on hydroxychloroquine, and induced with rituximab | Headache and vision changes resolved; remained stable after treatment | ||
| Vachvanichsanong et al (1992) []23 | Yes | Prednisolone (2 mg/kg/day), acetazolamide (125 mg 3 times a day) | Improved, with normal ophthalmologic findings and intracranial pressure | ||
| Donald et al (1973) []24 | Yes 1/4 | Prednisone or methylprednisolone therapy | Improved, with reduced intracranial pressure and resolved papilledema | ||
| Green et al (1995) []25 | Yes 1/3 | Prednisone, acetazolamide, IVIG, heparin, and warfarin | Improved, with no recurrence in most cases | ||
| Padeh & Passwell (1996) []26 | No | Steroids and azathioprine | Resolution, but relapse occurred | ||
| Yoo et al (2001) []27 | Yes | Prednisolone (60 mg/day initially, then tapered), hydroxychloroquine (300 mg/day maintenance) | Improved, with normal CSF opening pressure after 3 months; recurrence managed successfully | ||
| Komura et al (2002) []28 | Yes | High-dose corticosteroids (methylprednisolone), acetazolamide | Improved, with resolution of symptoms and normalization of intracranial pressure | ||
| Sbeiti et al (2003) []29 | Yes | Prednisone (1 mg/kg/day), acetazolamide (250 mg/day) | Improved, with resolution of symptoms and normalization of intracranial pressure | ||
| Kuyucu et al (2007) []30 | Yes | Methylprednisolone i.v. (1 g × 3 days) → oral prednisolone + azathioprin | Symptoms resolved, and papilledema disappeared in 3 weeks | ||
| Hershko et al (2008) []5 | Yes, all 10/10 | 9/10 treated with corticosteroids, 9/10 treated with acetazolamide, and 2/10 received azathioprine | 5/10 had complete remission, 2/10 had relapse, and 3/10 had permanent optic nerve damage | ||
| Kalanie et al (2019) []31 | Yes | Prednisolone (1 mg/kg/day), acetazolamide (250 mg/day) | Complete resolution of symptoms within 2 weeks; no recurrence during follow-up | ||
| Ang et al (2018) []32 | No | Corticosteroids and immunosuppressants (cyclophosphamide) | Resolution of symptoms, partial remission | ||
| Omer et al (2022) []33 | Yes | Prednisolone (1 mg/kg/day), hydroxychloroquine, acetazolamide (250 mg/day initially) | Complete recovery, with cessation of bleeding and normalization of laboratory parameters | ||
| Tse et al (2012) []34 | Yes | Prednisone (1 mg/kg/day), acetazolamide (250 mg/day), and hydroxychloroquine (200 mg twice daily) | Complete resolution of symptoms and normalization of intracranial pressure | ||
| Georgakopoulos et al 2011) []35 | Yes | Prednisolone (1 mg/kg/day) and acetazolamide (250 mg/day) | Complete resolution of symptoms and normalization of intracranial pressure | ||
| Cocco et al (2023) []36 | No | Not detailed in the study (focus on autoantibody identification) | Higher SLEDAI scores in NPSLE (13.9 vs 4.4 in SLE); grey matter hyperintensities on MRI associated with brain-reactive autoantibodies in NPSLE | ||
| Shah et al (2018) []37 | Yes | Prednisolone (1 mg/kg/day) and acetazolamide (250 mg/day) | Complete resolution of symptoms and normalization of intracranial pressure | ||
| Xue et al (2009) []14 | Yes | Prednisolone 80 mg/day, mannitol, glycerol + fructose, and cyclophosphamide | Significant improvement after 6 months | ||
| Laassila et al (2022) []38 | Yes | Pulse intravenous methylprednisolone (1 g/day for 5 days), followed by oral prednisone (1 mg/kg/day), hydroxychloroquine (400 mg/day), rituximab (500 mg every 6 months), acetazolamide (750 mg/day), and therapeutic lumbar puncture | Symptoms improved, and MRI lesions disappeared after 3 months of treatment | ||
| Silberberg & Laties (1973) []24 | Yes | Prednisone (60 mg/day) and acetazolamide | Complete resolution of symptoms and normalization of intracranial pressure | ||
| Shust et al (2024) []39 | Yes | Acetazolamide, topiramate, and azathioprine | Disc edema was nearly resolved | ||
| Srimanan et al (2022) []40 | Yes | Intravenous pulse methylprednisolone for 3 days then 1 mg/kg/day of oral prednisolone, with tapered dosage. For lupus nephritis: cyclophosphamide (600 mg) monthly for 6 doses with mycophenolate mofetil and corticosteroid | Papilledema improved. At 5-month follow-up, developed faint posterior subcapsular cataracts in both eyes to prolonged corticosteroid treatment, with best-corrected vision of 20/50 in both eyes | ||
| Our case | Yes | Furosemide 20 mg orally once daily; prednisone 30 mg orally twice daily; hydroxychloroquine 200 mg orally once daily; acetazolamide 250 mg orally twice daily; lisinopril 5 mg orally once daily; mycophenolate mofetil 1500 mg orally twice daily; and calcium carbonate/vitamin D (1500 mg/800 IU) orally once daily | Two weeks after discharge, pancytopenia and symptoms improved | ||