Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome
IntroductionMyelitis is the least common neuropsychiatric manifestation in systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI)-negative myelitis is even rarer. Here, we present the largest cohort of MRI-negative lupus myelitis cases to assess their clinical and immunological profile...
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Frontiers Media S.A.
2022-10-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2022.968322/full |
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| author | Shambaditya Das Biman Kanti Ray Arka Prava Chakraborty Abhirup Banerjee Alak Pandit Gautam Das Souvik Dubey |
| author_facet | Shambaditya Das Biman Kanti Ray Arka Prava Chakraborty Abhirup Banerjee Alak Pandit Gautam Das Souvik Dubey |
| author_sort | Shambaditya Das |
| collection | DOAJ |
| description | IntroductionMyelitis is the least common neuropsychiatric manifestation in systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI)-negative myelitis is even rarer. Here, we present the largest cohort of MRI-negative lupus myelitis cases to assess their clinical and immunological profiles and outcome.MethodA single-center, observational study conducted over a period of 5 years (2017–2021) was undertaken to evaluate patients with MRI-negative lupus myelitis for the epidemiological, clinical, immunological, and radiological features at baseline and followed up at monthly intervals for a year, and the outcomes were documented. Among the 22 patients that presented with MRI-negative myelopathy (clinical features suggestive of myelopathy without signal changes on spinal-cord MRI [3Tesla], performed serially at the time of presentation and 7 days, 6 weeks, and 3 months after the onset of symptoms), 8 patients had SLE and were included as the study population.ResultsIn 8 of 22 patients presenting with MRI-negative myelopathy, the etiology was SLE. MRI-negative lupus myelitis had a female preponderance (male: female ratio, 1:7). Mean age at onset of myelopathy was 30.0 ± 8.93 years, reaching nadir at 4.9 ± 4.39 weeks (Median, 3.0; range, 1.25–9.75). Clinically, cervical cord involvement was observed in 75% of patients, and 62.5% had selective tract involvement. The mean double stranded deoxyribonucleic acid, C3, and C4 titers at onset of myelopathy were 376.0 ± 342.88 IU/ml (median, 247.0), 46.1 ± 17.98 mg/dL (median, 47.5), and 7.3 ± 3.55 mg/dL (median, 9.0), respectively, with high SLE disease activity index 2,000 score of 20.6 ± 5.9. Anti-ribosomal P protein, anti-Smith antibody, and anti-ribonuclear protein positivity was observed in 87.5, 75, and 75% of the patients, respectively. On follow-up, improvement of myelopathic features with no or minimal deficit was observed in 5 of the 8 patients (62.5%). None of the patients had recurrence or new neurological deficit over 1-year follow-up.ConclusionPersistently “MRI-negative” lupus myelitis presents with white matter dysfunction, often with selective tract involvement, in light of high disease activity, which follows a monophasic course with good responsiveness to immunosuppressive therapy. A meticulous clinical evaluation and a low index of suspicion can greatly aid in the diagnosis of this rare clinical condition in lupus. |
| first_indexed | 2024-04-11T08:53:37Z |
| format | Article |
| id | doaj.art-1101d84f7cc342ac830c82388f131ad7 |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-11T08:53:37Z |
| publishDate | 2022-10-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Neurology |
| spelling | doaj.art-1101d84f7cc342ac830c82388f131ad72022-12-22T04:33:21ZengFrontiers Media S.A.Frontiers in Neurology1664-22952022-10-011310.3389/fneur.2022.968322968322Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcomeShambaditya Das0Biman Kanti Ray1Arka Prava Chakraborty2Abhirup Banerjee3Alak Pandit4Gautam Das5Souvik Dubey6Department of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, IndiaDepartment of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, IndiaDepartment of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, IndiaDepartment of General Medicine, Institute of Post Graduate Medical Education & Research, Kolkata, IndiaDepartment of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, IndiaDepartment of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, IndiaDepartment of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, IndiaIntroductionMyelitis is the least common neuropsychiatric manifestation in systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI)-negative myelitis is even rarer. Here, we present the largest cohort of MRI-negative lupus myelitis cases to assess their clinical and immunological profiles and outcome.MethodA single-center, observational study conducted over a period of 5 years (2017–2021) was undertaken to evaluate patients with MRI-negative lupus myelitis for the epidemiological, clinical, immunological, and radiological features at baseline and followed up at monthly intervals for a year, and the outcomes were documented. Among the 22 patients that presented with MRI-negative myelopathy (clinical features suggestive of myelopathy without signal changes on spinal-cord MRI [3Tesla], performed serially at the time of presentation and 7 days, 6 weeks, and 3 months after the onset of symptoms), 8 patients had SLE and were included as the study population.ResultsIn 8 of 22 patients presenting with MRI-negative myelopathy, the etiology was SLE. MRI-negative lupus myelitis had a female preponderance (male: female ratio, 1:7). Mean age at onset of myelopathy was 30.0 ± 8.93 years, reaching nadir at 4.9 ± 4.39 weeks (Median, 3.0; range, 1.25–9.75). Clinically, cervical cord involvement was observed in 75% of patients, and 62.5% had selective tract involvement. The mean double stranded deoxyribonucleic acid, C3, and C4 titers at onset of myelopathy were 376.0 ± 342.88 IU/ml (median, 247.0), 46.1 ± 17.98 mg/dL (median, 47.5), and 7.3 ± 3.55 mg/dL (median, 9.0), respectively, with high SLE disease activity index 2,000 score of 20.6 ± 5.9. Anti-ribosomal P protein, anti-Smith antibody, and anti-ribonuclear protein positivity was observed in 87.5, 75, and 75% of the patients, respectively. On follow-up, improvement of myelopathic features with no or minimal deficit was observed in 5 of the 8 patients (62.5%). None of the patients had recurrence or new neurological deficit over 1-year follow-up.ConclusionPersistently “MRI-negative” lupus myelitis presents with white matter dysfunction, often with selective tract involvement, in light of high disease activity, which follows a monophasic course with good responsiveness to immunosuppressive therapy. A meticulous clinical evaluation and a low index of suspicion can greatly aid in the diagnosis of this rare clinical condition in lupus.https://www.frontiersin.org/articles/10.3389/fneur.2022.968322/fullmyelitis in lupusMRI-negative myelitisMRI-negative lupus myelitissystemic lupus erythematosusneuropsychiatric systemic lupus erythematosusselective tractopathy |
| spellingShingle | Shambaditya Das Biman Kanti Ray Arka Prava Chakraborty Abhirup Banerjee Alak Pandit Gautam Das Souvik Dubey Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome Frontiers in Neurology myelitis in lupus MRI-negative myelitis MRI-negative lupus myelitis systemic lupus erythematosus neuropsychiatric systemic lupus erythematosus selective tractopathy |
| title | Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome |
| title_full | Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome |
| title_fullStr | Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome |
| title_full_unstemmed | Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome |
| title_short | Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome |
| title_sort | persistent mri negative lupus myelitis disease presentation immunological profile and outcome |
| topic | myelitis in lupus MRI-negative myelitis MRI-negative lupus myelitis systemic lupus erythematosus neuropsychiatric systemic lupus erythematosus selective tractopathy |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2022.968322/full |
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