scholarly journals Systemic lupus erythematosus with the development of neuromyelitis optica (Devic's syndrome) is a rare combination of autoimmune diseases

2019 ◽  
Vol 13 (4) ◽  
pp. 89-95 ◽  
Author(s):  
E. S. Vinogradova ◽  
A. P. Panova ◽  
N. M. Bulanov ◽  
P. I. Novikov ◽  
S. V. Moiseev
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Demyelinating Disease ◽  
Transverse Myelitis ◽  
Young Female ◽  
Systemic Lupus ◽  
Devic’S Syndrome ◽  
Immune Mediated

Neuromyelitis optica ((NMO), Devic's syndrome) is an immune-mediated inflammatory demyelinating disease characterized by transverse myelitis and optic neuritis. Determination of the level of antibodies to aquaporin 4 (NMO-IgG) is presently one of the key methods for the diagnosis and assessment of the activity of ONM, which allows this disease to be differentiated from multiple sclerosis and other demyelinating CNS lesions. ONM can occur not only as an independent disease, but also as a syndrome in different systemic diseases, such as: systemic lupus erythematosus (SLE), antineutrophilic cytoplasmic antibody-associated vasculitides, Sjögren's disease, etc. (up to 50–70%). In such situations, the clinician is always confronted with a question as whether the patient can have two rare autoimmune diseases or develop ONM as a systemic manifestation of rheumatic disease.The paper describes a clinical case of a young female patient with SLE concurrent with a CNS lesion, the manifestations of which corresponded to ONM. The patient had focal changes in the substance of the brain and spinal cord, as evidenced by magnetic resonance imaging, as well as high NMO-IgG titers. The development of ONM worsens SLE prognosis and requires active immunosuppressive therapy. The patient received three plasmapheresis sessions, ultrahigh-dose glucocorticoid and cyclophosphamide therapy, followed by replacement with azathioprine, causing a stable clinical and laboratory disease remission to be achieved.

scholarly journals Systemic Lupus Erythematosus (SLE) Complicated by Neuromyelitis Optica (NMO – Devic's Disease): Clinic-Pathological Report and Review of the Literature

2014 ◽  
Vol 7 ◽  
pp. CCRep.S15177 ◽  
Author(s):  
Mohammad Adawi ◽  
Bishara Bisharat ◽  
Abdalla Bowirrat
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Demyelinating Disease ◽  
Clinical Laboratory ◽  
Transverse Myelitis ◽  
Systemic Lupus ◽  
Igg Antibody ◽  
Laboratory Findings

Neuromyelitis optica (NMO) is usually a relapsing demyelinating disease of the central nervous system associated with optic neuritis, transverse myelitis involving three or more contiguous spinal cord segments, and seropositivity for NMO-IgG antibody. NMO is often mistaken for multiple sclerosis and there are relatively sporadic publications about NMO and overlapping systemic or organ-specific autoimmune diseases, such as systemic lupus erythematosus (SLE). We described a unique case of a 25-year-old Arab young woman who was diagnosed with SLE, depending on clinical, laboratory investigations and after she had fulfilled the diagnostic criteria for SLE and had presented the following findings: constitutional findings (fatigue, fever, and arthralgia); dermatologic finding (photosensitivity and butterfly rash); chronic renal failure (proteinuria up to 400 mg in 24 hours); hematologic and antinuclear antibodies (positivity for antinuclear factor (ANF), anti-double-stranded DNA antibodies, direct Coombs, ANA and anti-DNA, low C4 and C3, aCL by IgG and IgM). Recently, she presented with several episodes of transverse myelitis and optic neuritis. Clinical, radiological, and laboratory findings especially seropositivity for NMO-IgG were compatible with NMO. Accurate diagnosis is critical to facilitate initiation of immunosuppressive therapy for attack prevention. This case illustrates that NMO may be associated with SLE.

Neuromyelitis optica spectrum disorder and systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1722-1726 ◽  
Author(s):  
M M Thabah ◽  
Sekar D ◽  
R Pranov ◽  
M M V Moulitej ◽  
A Ramesh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Systemic Lupus ◽  
Autoimmune Conditions ◽  
Inflammatory Syndrome ◽  
Extensive Transverse Myelitis

Neuromyelitis optica spectrum disorder is an inflammatory syndrome that is associated with many autoimmune conditions. We present the case of a patient who had longitudinally extensive transverse myelitis and antibodies to aquaporin 4 IgG (AQP4-IgG). Based on presence of lymphopenia, further workup revealed strong ANA positivity, anti-Sm antibodies, and low serum complements suggesting presence of systemic lupus erythematosus. The patient promptly responded to intravenous pulse methylprednisolone and five sessions of plasma exchange. At 1 year, she is on maintenance treatment with low dose prednisolone, azathioprine, and hydroxychloroquine, she has had no relapse and no other clinical features of lupus. This case is an illustration that neuromyelitis optica spectrum disorder can be the first manifestation of systemic lupus erythematosus.

Neuromyelitis optica (Devic’s syndrome) as first manifestation of systemic lupus erythematosus

Lupus ◽  
2006 ◽  
Vol 15 (2) ◽  
pp. 107-109 ◽  
Author(s):  
C Jacobi ◽  
K Stingele ◽  
R Kretz ◽  
M Hartmann ◽  
B Storch-Hagenlocher ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Devic’S Syndrome

Neuromyelitis optica spectrum disorder in a patient with systemic lupus erythematosus and anti-phospholipid antibody syndrome

2008 ◽  
Vol 14 (3) ◽  
pp. 425-427 ◽  
Author(s):  
Lahar R Mehta ◽  
Melissa K Samuelsson ◽  
Anatole K Kleiner ◽  
Andrew D Goodman ◽  
Jennifer H Anolik ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Specific Marker ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
History Of ◽  
Recurrent Transverse Myelitis

Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system characterized by severe episodes of optic nerve and spinal cord inflammation. NMO-IgG (anti-aquaporin-4) has been recently described as a sensitive and specific marker for NMO. As there have been prior published reports of an association between NMO and systemic autoimmune diseases, the prognostic value of the antibody test in these cases is uncertain. We describe a 47-year old woman with recurrent transverse myelitis and a long-standing history of systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APLS). While she did not have a history of optic neuritis, serological testing for the NMO-IgG was positive when she was admitted for her second episode of transverse myelitis. Testing for the NMO-IgG in cases of isolated or recurrent transverse myelitis attributed to current SLE and APLS may help clarify the diagnosis of a distinct disease process likely to cause recurrent and severe disability, warranting more aggressive immunotherapy. Multiple Sclerosis 2008; 14: 425—427. http://msj.sagepub.com

scholarly journals Neuromyelitis optica spectrum disorder in patient with systemic lupus erythematosus - our experience

2018 ◽  
Vol 75 (1) ◽  
pp. 104-107
Author(s):  
Ksenija Bozic ◽  
Nenad Komatina ◽  
Milan Petronijevic ◽  
Bojana Knezevic ◽  
Dejan Kostic ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Patient Treatment ◽  
Caucasian Woman ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Systemic Lupus ◽  
Longitudinal Extensive Transverse Myelitis

Introduction. Neuromyelitis optica spectrum disorder (NMOSD) is a rare demyelinating immune-mediated central nervous system disease. It is extremely rare to occur in patients with systemic lupus erythematosus (SLE), and it represents a diagnostic and therapeutic challenge. Case report. A 38-year-old Caucasian woman with medical history of SLE and new onset of flaccid paraparesis, fecal and urinary incontinence, persistent nausea and vomiting was admitted to our hospital. Based on the clinical presentation, magnetic resonance imaging findings and positive aquaporin 4 (AQP4) antibodies, a NMOSD with coexisting SLE were diagnosed. Pulse-doses of cyclophosphamide and glucocorticoids were efficient in patient treatment. Conclusion. In a patient with SLE and symptoms of longitudinal extensive transverse myelitis and/or optic neuritis and area postrema syndrome, assessment of AQP4 antibodies is neccessary for diagnosing NMOSD. Accurate diagnosis, and timely and long-term administration of immunosuppressive therapy are crucial for favorable outcome of these two coexisting diseases.

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