Is It Lupus? Is It Neuromyelitis Optica Spectrum Disorder (NMOSD)?—Why Not Both?
Abstract
:1. Introduction
2. Materials and Methods
2.1. Laboratory Analysis
2.2. MR-Imaging
2.3. Neurophysiological Diagnostic
2.4. Diagnostic Flow
3. Case Presentation
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Parameter | Value | Reference Values |
---|---|---|
CSF | ||
cell count | 20/µL | <5/µL |
protein | 22 mg/dL | 15–45 mg/dL |
lactate | 2.3 mmol/L | 1.1–2.4 mmol/L |
glucose | 65 mg/dL | n.a. |
hemoglobin | negative | negative |
blood/serum | ||
IL2-receptor | 715 U/mL | 158–623 U/mL |
ANA | 1:640 | <1:80 |
SSA/Ro Antibodies | 11 U/mL | <7 U/mL |
dsDNA antibodies | 5.2 U/mL | <10 U/mL |
86 U/mL * | ||
cardiolipin IgG | 11 U/mL | <10 U/mL |
cardiolipin IgM | 28 U/mL | <10 U/mL |
β2-Glycoprotein IgG | 14 U/mL | <7 U/mL |
β2-Glycoprotein IgM | 20 U/mL | <7 U/mL |
C3c | 80.1 mg/dL | 90–180 mg/dL |
C4 | 8.0 mg/dL | 10–40 mg/dL |
hemoglobin | 12.4 g/dL | 12.0–16.0 g/dL |
5.2 g/dL * | ||
thrombocytes | 163,000/µL | 140,000–400,000/µL |
63,000/µL | ||
creatinine | 0.59 mg/dL | 0.5–0.9 mg/dL |
anti-Sm antibodies | 1.3 U/mL | <7 |
AQP4 antibodies | 1:10,240 | <1:80 |
anti-MOG antibodies | negative | negative |
Entry Criterion: Antinuclear Antibodies (ANA) at a Titer of ≥1:80 on HEp-2 Cells or an Equivalent Positive Test (Ever) | ||
---|---|---|
Additive Criteria: | ||
Clinical Domains and Criteria | Weight | Present in the Patient |
Constitutional • Fever | 2 | |
Hematologic • Leukopenia • Thrombocytopenia • Autoimmune hemolysis | 3 4 4 | X X |
Neuropsychiatric • Delirium • Psychosis • Seizure | 2 3 5 | |
Mucocutaneous • Non-scarring alopecia • Oral ulcers • Subacute cutaneous or discoid lupus • Acute cutaneous lupus | 2 2 4 6 | |
Serosal • Pleural or pericardial effusion • Acute pericarditis | 5 6 | |
Musculoskeletal • Joint involvement | 6 | |
Renal • Proteinuria > 0.5 g/24 h • Renal biopsy class II or V lupus nephritis • Renal biopsy class III or IV lupus nephritis | 4 8 10 | |
Immunology domains and criteria | ||
Antiphospholipid antibodies • Anti-cardiolipin antibodies OR • Anti-β2GP1 antibodies OR • Lupus anticoagulant | 2 | X |
Complement proteins • Low C3 OR low C4 • Low C3 AND low C4 | 3 4 | X |
SLE-specific antibodies • Anti-dsDNA antibody OR • Anti-Smith antibody | 6 | X |
Classification as SLE with a score of 10 or more combined with fulfilled entry criterion. Occurrence of a criterion on at least 1 occasion is sufficient. Criteria do not have to occur simultaneously |
Diagnostic Criteria for NMOSD with AQP4-IgG | |
---|---|
1. At least 1 core clinical characteristic of the following: | 1. Optic neuritis 2. Acute myelitis 3. Area postrema syndrome episode: episode of otherwise unexplained hiccups or nausea and vomiting 4. Acute brainstem syndrome 5. Symptomatic narcolepsy or acute diencephalic syndrome with NMOSD-typical diencephalic MRI lesions 6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions |
2. Positive test for AQP4-IgG using best available detection method (cell-based assay strongly recommended) | |
3. Exclusion of alternative diagnoses |
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© 2023 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
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Kaempfer, N.A.; Fousse, M.; Kettner, M.; Fassbender, K.; Janitschke, D. Is It Lupus? Is It Neuromyelitis Optica Spectrum Disorder (NMOSD)?—Why Not Both? Sclerosis 2023, 1, 51-59. https://doi.org/10.3390/sclerosis1010006
Kaempfer NA, Fousse M, Kettner M, Fassbender K, Janitschke D. Is It Lupus? Is It Neuromyelitis Optica Spectrum Disorder (NMOSD)?—Why Not Both? Sclerosis. 2023; 1(1):51-59. https://doi.org/10.3390/sclerosis1010006
Chicago/Turabian StyleKaempfer, Niklas Alexander, Mathias Fousse, Michael Kettner, Klaus Fassbender, and Daniel Janitschke. 2023. "Is It Lupus? Is It Neuromyelitis Optica Spectrum Disorder (NMOSD)?—Why Not Both?" Sclerosis 1, no. 1: 51-59. https://doi.org/10.3390/sclerosis1010006