Neuropathy In Systemic Lupus Erythematosus Research Articles

Clinical, histologic, and immunologic signatures of Small Fiber Neuropathy in Systemic Lupus Erythematosus.

Systemic Lupus Erythematosus (SLE) often causes damage to small nerve fibers, leading to distressing painful and autonomic symptoms. Despite this, Small Fiber Neuropathy (SFN) remains an underrecognized complication for SLE patients. In this cross-sectional study, we aimed to assess SFN in patients with SLE and to explore its correlations with immunologic disease features and clinical manifestations. We recruited 50 SLE patients (1 male to 12.5 females, aged 20-80 years) reporting painful disturbances. We conducted a comprehensive clinical and neurophysiological evaluation, using Nerve Conduction Studies and Quantitative Sensory Testing. Additionally, we carried out an extensive laboratory assessment of disease-related serological parameters. We also performed a thorough skin biopsy analysis, investigating somatic and autonomic innervation while detecting complement and inflammatory cell infiltrates within the skin. Out of 50 patients, 19 were diagnosed with SFN, primarily characterized by a non-length-dependent distribution; 7 had a mixed neuropathy, with both large and small fiber involvement. Patients with SFN were younger than patients with a mixed neuropathy (p = .0143); furthermore, they were more likely to have a history of hypocomplementemia (p = .0058) and to be treated with cyclosporine A (p = .0053) compared to patients without neuropathy. However, there were no significant differences in painful and autonomic symptoms between patients with and without SFN. This study highlights the relevant frequency of SFN with a non-length-dependent distribution among SLE patients experiencing painful symptoms. Indeed, SFN emerges as an early manifestation of SLE-related neuropathy and is closely associated with hypocomplementemia, suggesting a potential pathogenic role of the complement system. Moreover, SFN may be influenced by disease-modifying therapies. However, the precise role of SFN in shaping painful and autonomic symptoms in patients with SLE remains to be fully elucidated.

Anti-KIF20B autoantibodies are associated with cranial neuropathy in systemic lupus erythematosus

BackgroundCranial neuropathies (CN) are a rare neuropsychiatric SLE (NPSLE) manifestation. Previous studies reported that antibodies to the kinesin family member 20B (KIF20B) (anti-KIF20B) protein were associated with idiopathic ataxia and...

Systemic lupus erythematosus with acute ischemic optic neuropathy complicated with neuromyelitis optica: a case report

BackgroundNeuromyelitis optica is a relapsing–remitting disease characterized by a recurrent attack of optic neuritis and transverse myelitis; sometimes associated with acute brainstem syndrome. Systemic lupus erythematosus is an autoimmune multisystem disorder in which ocular involvement such as acute ischemic optic neuropathy is a rare manifestation. However, neuromyelitis optica can be associated with systemic lupus erythematosus.Case presentationA 24-year-old Bangladeshi woman was admitted to the hospital with complaints of sudden, progressive, painless vision loss in both eyes, and progressive weakness in both lower limbs for 48 hours. She also gave a history of arthralgia, a photosensitive skin rash, intermittent fever, oral ulcerations, and alopecia for the last 2 months. On examination, the fundus was suggestive of bilateral acute ischemic neuropathy, and examinations of the lower limb revealed spastic paraparesis with sensory abnormality. Laboratory investigations revealed the presence of positive anti-aquaporin 4 antibody, strongly positive antinuclear antibody, and anti-ds DNA with the longitudinally extensive lesion on magnetic resonance imaging of the spinal cord. She was treated with methylprednisolone, hydroxychloroquine, and mycophenolate, and was discharged with improvement of her paraparesis. However, her vision did not improve substantially.ConclusionThe importance of this report is to shed some light on the occurrence of two devastating complications that is, bilateral acute ischemic optic neuropathy in systemic lupus erythematosus complicated by neuromyelitis optica, as well as evidence of rare presentations for systemic lupus erythematosus and treatment modalities of ischemic optic neuropathy with systemic lupus erythematosus.

Peripheral neuropathy in systemic lupus erythematosus: what can neuromuscular ultrasonography (NMUS) tell us? A cross-sectional study

ObjectiveTo evaluate peripheral nerve involvement in patients with SLE with neuromuscular ultrasonography (NMUS) and understand its role in investigating SLE-related peripheral neuropathy.MethodsThis is an observational cross-sectional study on patients with...

Pattern of peripheral neuropathy in systemic lupus erythematosus: clinical, electrophysiological, and laboratory properties and their association with disease activity

AimTo study clinical, electrophysiological, and laboratory properties of peripheral neuropathy (PN) in systemic lupus erythematosus (SLE) and their association with disease activity.Patients and methodsA totalof 30 patients who met the American College of Rheumatology case definition criteria for SLE-PN and 30 age-matched and sex-matched patients with SLE without PN were selected from the Main Alexandria University Hospital Physical Medicine, Rheumatology and Rehabilitation clinic. Demographic data, SLE-related clinical, laboratory data, Systemic Lupus Activity Measure (SLAM) index, and nerve conduction studies were done. This case–control study compared clinical and SLE-related features, laboratory, and SLAM index in patients with SLE with PN versus those without neuropathy.ResultsThe results showed that the most common PN subtype was sensorimotor polyneuropathy which occurred in 18 (60%) patients; the most common PN pathology was axonal degeneration, which occurred 19 (63.3%) patients; and the most common associated nerve entrapment was carpal tunnel syndrome in 10 (33.3%) patients. In comparison between group I (SLE with PN) and group II (SLE without PN), there was no statistically significant difference between the two groups regarding demographic data, disease duration, and lupus clinical features, except malar rash and lupus nephritis, which showed significant increase in patients with SLE with PN compared with patients with SLE without PN (P=0.003 and P<0.001, respectively). There was no statistically significant difference among PN subtype groups regarding sex, age, and immunological markers. Regarding diseases activity, SLAM index showed a significant increase in patients with SLE with PN compared with patients with SLE without PN (P=0.006).ConclusionThe pattern of neuropathy in SLE is mainly axonal. Moreover, the most common PN subtype is sensorimotor polyneuropathy. The study suggests significant association of PN in patients with SLE with nephritis, malar rash, and SLAM index.

Monocular Oculomotor Nerve Disorder Manifesting as Cranial Neuropathy in Systemic Lupus Erythematosus

We herein report the case of a patient who developed peripheral neuropathy of the bilateral lower legs that later became complicated with isolated oculomotor nerve disorder and was finally diagnosed as systemic lupus erythematosus (SLE). Based on the findings for oculomotor nerve paralysis and contrast-enhanced magnetic resonance imaging findings for the oculomotor nerve in the prepontine cistern, the isolated oculomotor nerve disorder was considered to be a manifestation of peripheral neuropathy. This oculomotor nerve disorder may contribute to the diagnosis of SLE and can be effectively treated with steroid pulse therapy. Reports of SLE manifesting as isolated oculomotor nerve paralysis are rare.

Upper extremity subclinical autonomic and peripheral neuropathy in systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune, multiorgan disease that affects connective tissues of many organs or systems, including the nervous system, where it affects the autonomic, the peripheral, and the central nervous system. The aim of this study was to investigate the association of subclinical autonomic and peripheral neuropathy with SLE and to correlate neurophysiological parameters with clinical and laboratory data. Fifty-six SLE patients were included in this study. In addition, thirty age-matched and sex-matched healthy participants served as a control group. Exclusion criteria included patients having symptoms or signs indicating autonomic dysfunction or peripheral neuropathy. Also, endocrinal, toxic, compression, and traumatic neuropathies were excluded. Patients were assessed clinically and by laboratory investigations. Neurophysiological assessment included sympathetic skin response of the median nerve including latency and amplitude. In addition, nerve conduction study of both median and ulnar nerves was performed including motor distal latency, amplitude, nerve conduction velocity, and distal sensory latency. Pure sensory abnormality was detected in one patient, whereas pure motor neuropathy was found in 19 patients. Mixed sensory–motor abnormalities were detected in two patients. Sympathetic skin response was not elicited in 13 patients, whereas latency and amplitude abnormalities were detected in 11/43 and 9/43 patients, respectively. Sympathetic and axonal neuropathy was not correlated with the disease duration or the disease activity. The pattern of neuropathy in SLE is mainly axonal. Also, the sympathetic nervous system is affected in lupus patients with a rate of up to 40% of the cases.

Clinical and electrophysiological characteristics of symmetric polyneuropathy in a cohort of systemic lupus erythematosus patients.

Peripheral neuropathy in systemic lupus erythematosus (SLE) is heterogeneous and its commonest pattern is symmetrical polyneuropathy. The aim of this study was to describe the prevalence, clinical and electrophysiological features, disease associations and effects on function and quality of life of polyneuropathy in SLE patients, defined using combined clinical and electrophysiological diagnostic criteria. Consecutive SLE patients seen at the University of Malaya Medical Centre were included. Patients with medication and other disorders known to cause neuropathy were excluded. Demographic, clinical and laboratory data were obtained using a pre-defined questionnaire. Function and health-related quality of life was assessed using the modified Rankin scale and the SF-36 scores. Nerve conduction studies (NCS) were carried out in both upper and lower limbs. Polyneuropathy was defined as the presence of bilateral clinical symptoms and/or signs and bilateral abnormal NCS parameters. Of 150 patients, 23 (15.3%) had polyneuropathy. SLE-related polyneuropathy was mainly characterized by sensory symptoms of numbness/tingling and pain with mild signs of absent ankle reflexes and reduced pain sensation. Function was minimally affected and there were no differences in quality of life scores. NCS abnormalities suggested mild length-dependent axonal neuropathy, primarily in the distal lower limbs. Compared to those without polyneuropathy, SLE-related polyneuropathy patients were significantly older but had no other significant demographic or disease associations. SLE-related polyneuropathy is a chronic, axonal and predominantly sensory neuropathy, associated with older age. Its underlying pathogenetic mechanisms are unknown, although a possibility could be an increased susceptibility of peripheral nerves in SLE patients to effects of aging.

Sensory neuronopathy complicating systemic lupus erythematosus: a case report

IntroductionSystemic lupus erythematosus is a multi-system connective tissue disorder. Peripheral neuropathy is a known and underestimated complication in systemic lupus erythematosus. Ganglionopathy manifests when neuronal cell bodies in the dorsal root ganglion are involved. Autoimmune disorders are a known etiology, with systemic lupus erythematosus being a rare cause.Case presentationA 32-year-old South Asian woman presented with oral ulceration involving her lips following initiation of treatment for a febrile illness associated with dysuria. She had a history of progressively worsening numbness over a period of 4 months involving both the upper and lower limbs symmetrically while sparing the trunk. Her vibration sense was impaired, and her reflexes were diminished. For the past 4 years, she had had a bilateral, symmetrical, non-deforming arthritis involving the upper and lower limbs. Her anti-nuclear antibody and anti-double-stranded deoxyribonucleic acid status were positive. Although her anti-Ro antibodies were positive, she did not have clinical features suggestive of Sjögren syndrome. Nerve conduction studies revealed sensory neuronopathy. A diagnosis of systemic lupus erythematosus complicated by sensory neuronopathy was made. Treatment with intravenous immunoglobulin resulted in clinical and electrophysiological improvement.ConclusionPeripheral neuropathy in systemic lupus erythematosus can, by itself, be a disabling feature. Nerve conduction studies should be considered when relevant. Neuropathy in systemic lupus erythematosus should be given greater recognition, and rarer forms of presentation should be entertained in the differential diagnosis when the clinical picture is atypical. Intravenous immunoglobulin may have role in treatment of sensory neuronopathy in systemic lupus erythematosus.

Concomitant occurrence of cervical myelopathy, cerebral infarction, and peripheral neuropathy in systemic lupus erythematosus: a case report.

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease characterized by multiorgan involvement with diverse clinical presentations. Central nervous system involvement in neuropsychiatric syndromes of SLE (NPSLE), such as cerebrovascular disease and myelopathy, is a major cause of morbidity and mortality in SLE patients. The concomitant occurrence of myelopathy, cerebrovascular disease, and peripheral neuropathy in a patient with SLE has not yet been reported. We report on a 41-year-old woman with SLE who showed motor and sensory impairment with urinary retention and was diagnosed with cervical myelopathy and acute cerebral infarction by spine and brain magnetic resonance imaging and peripheral neuropathy by electrodiagnostic examination. Even though pathogenesis of NPSLE is not well elucidated, we assume that increased antibodies of anti-double stranded DNA (anti-dsDNA), presence of lupus anticoagulant and hypertension are risk factors that have caused neuropsychiatric lupus in this patient.

Cranial nerve VI palsy as a rare initial presentation of systemic lupus erythematosus: case report and review of the literature

A 48-year-old woman presented with isolated sixth cranial nerve palsy. She subsequently developed systemic lupus erythematosus (SLE) based on clinical and laboratory parameters. Three years later, she presented again with sixth cranial nerve palsy affecting the contralateral eye. Within 2 weeks of steroid initiation, complete recovery occurred. The unusual rare presentation of SLE in the current patient, as well as the pathogenesis and treatment of cranial neuropathy in SLE are discussed.

Peripheral neuropathy in systemic lupus erythematosus: pathomorphological features and distribution pattern of matrix metalloproteinases.

Matrix metalloproteinases (MMPs) are endoproteases that have been implicated in the pathogenesis of inflammatory and vasculitic neuropathies. In systemic lupus erythematosus (SLE), a peripheral neuropathy is frequently seen that is thought to be caused by ischemic nerve damage due to vasculopathy and/or vasculitis of the nutritional vessels. However, the exact pathomechanisms causing SLE neuropathy are largely unknown. Elevated MMP levels have been reported in the serum of SLE patients. Supposing that altered expression of MMPs may contribute to vessel wall damage in SLE neuropathy, we investigated the expression of MMP-1, -2, -3, -9, -10 and -13, and their tissue inhibitors (TIMP-1 and -2) in sural nerves from 12 SLE patients in comparison to normal controls. All MMPs could be detected within blood vessel walls from SLE nerves, whereas in controls MMP-3 and MMP-9 was not found. TIMP-1 and TIMP-2, on the other hand, were not informative. Generally, small and large nutritional vessels in the epineurium were immunoreactive for MMPs and TIMPs. Mononuclear cells, which expressed MMP-1, - 3, -10, -13, and TIMP-1 were also observed in most of the SLE nerves, mostly around epineurial blood vessels, but only occasionally in controls. This indicates that expression of MMPs in mononuclear cells may be related to leukocyte trafficking through the vessel walls. However, the density of TIMP-positive and MMP-positive inflammatory cells did not correlate with morphometric parameters regarding the severity of the neuropathy. Our findings suggest that especially the up-regulation of MMP-3 and MMP-9 within the vessel walls may be responsible for the vascular damage seen in SLE and the resulting chronic combined axonal and demyelinating type of neuropathy frequently found in SLE.

Recovery After Two Separate Accidental Burn Injuries

A 19-year-old woman with systemic lupus erythematosus (SLE) developed Guillain-Barre like ascending motor paralysis with sensory neuropathy and was subsequently documented to have "necrotizing vasculitis" by sural nerve biopsy. After 1 month of aggressive treatment with high-dose corticosteroids, plasmapheresis, i.v. gamma globulin and cyclophosphamide, there was limited improvement in her neurologic examination. The patient was discharged with bilateral foot-drop and complete sensory loss in the lower legs. There was no improvement in her neurologic status for several months until she had two separate accidental burn injuries, first in the right leg then in the left, after which the motor and sensory function in each leg began to rapidly recover. This case not only illustrates an unusual pattern of neuropathy in SLE but also allows us to postulate that thermal injury may effect the function of stunned nerves by impairing immune function.

Peripheral neuropathy in systemic lupus erythematosus.

The prevalence of clinically apparent peripheral neuropathy in systemic lupus erythematosus is reported to be between 2% to 18%. The purpose of this prospective case-control study was to determine the prevalence of peripheral neuropathy (PN) using electrodiagnostic criteria. Subgroup analysis was performed to determine whether PN correlated with disease activity, renal involvement, or serum immune markers. Fifty-four systemic lupus erythematosus patients and 30 controls were recruited in the study. The right median, ulnar, peroneal, tibial, and sural sensory and motor nerve conduction studies were obtained. PN in our study was defined as any abnormal values in motor and sensory distal latency, sensory action potential, motor action potential, or conduction velocity affecting 2 or more nerves. Of the 54 patients studied, PN was present in 15 patients (27.8%) of which 4 were symptomatic. There was a significant correlation between PN and anti-SM antibody, and there was a trend showing decreased motor and sensory action potential amplitudes in our systemic lupus erythematosus group compared to the controls. This observation was also seen in an active disease group when compared to those with inactive disease. The amplitude of the action potential was more often affected than the distal latency, and sensory nerves were more susceptible than motor nerves. The sural sensory action potential amplitude appears to be the most sensitive indicator of PN which may be used as an index to monitor disease activity.

Peripheral neuropathy in systemic lupus erythematosus.

We studied 33 consecutive patients with systemic lupus erythematosus (SLE) for neuropathy by employing the Neuropathy Symptom Score (NSS), Neurological Disability Score (NDS), EMG and nerve conduction velocity (NCV) studies, and determinations of vibration thresholds (VT). Polyneuropathy defined as NCV abnormalities of two or more nerves occurred in seven patients (21%). Neuropathic symptoms showed a poor correlation with NCV and VT, while clinical neuropathic signs, VT, and NCV correlated with each other in most instances. When reporting frequencies of neuropathy in SLE, NCV studies should be used as a basis. NSS, NDS, and VT give additional quantifiable information and can be useful in the follow-up of patients and for evaluating the response to therapy.

Optic Neuropathy in Systemic Lupus Erythematosus

We report data from seven cases of optic neuropathy in systemic lupus erythematosus. The visual outcome varied, but improvement occasionally occurred following treatment with corticosteroids. The clinical picture was variable and could present as acute retrobulbar optic neuritis, ischemic optic neuropathy, or slowly progressive visual loss. Visual evoked response testing revealed increased latency in two patients and loss of amplitude in two others; a fifth patient had both findings in the involved eye. Despite the variable presentation, the probable pathogenesis in all cases is vaso-occlusive disease in small vessels of the optic nerves.