Intravenous Cyclophosphamide Treatment for Systemic Lupus Erythematosus with Severe Autonomic Disorders Confirmed by Head-Up Tilt Table Test: A Case Series

Abstract Autonomic disorders are common in patients with SLE, but the therapeutic strategy and methods for evaluating the effects of therapy have not been established. We describe the three cases of SLE patients who developed severe autonomic disorders as demonstrated by the head-up tilt table test (HUT). All three patients were treated by intensive immunosuppressive treatments including cyclophosphamide (IVCY); their HUT results all became negative. Our cases suggest that IVCY treatment can be a good therapeutic option for severe autonomic disorders in SLE patients. The HUT is a useful objective method for the diagnosis of and the evaluation of longitudinal therapeutic effects on autonomic disorders in SLE patients with orthostatic intolerance.

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Orthostatic Headaches Associated With Spontaneous Intracranial Hypotension and Autonomic Dysfunction—A Case Series in Young Patients

Child Neurology Open ◽

10.1177/2329048x211056709 ◽

2021 ◽

Vol 8 ◽

pp. 2329048X2110567

Author(s):

Ankita Ghosh ◽

Yen X. Tran ◽

Leon Grant ◽

Mohammed T. Numan ◽

Rajan Patel ◽

...

Keyword(s):

Autonomic Dysfunction ◽

Intracranial Hypotension ◽

Orthostatic Intolerance ◽

Spontaneous Intracranial Hypotension ◽

Young Patients ◽

Case Series ◽

Tilt Table ◽

Epidural Blood Patching ◽

Persistent Symptoms ◽

Head Up Tilt

Background: Orthostatic headaches can be noted in spontaneous intracranial hypotension and orthostatic intolerance. We present a case series of young patients diagnosed with spontaneous intracranial hypotension and were treated for the same but subsequently developed orthostatic intolerance. Methods: We retrospectively reviewed charts for seven young patients with orthostatic headaches related to spontaneous intracranial hypotension and orthostatic intolerance. Results: Patients were diagnosed with spontaneous intracranial hypotension. Diagnosis was confirmed by identifying epidural contrast leakage and three of seven patients were noted to have early renal contrast excretion on computerized tomography myelography. Patients were treated with epidural blood patches. All patients showed persistent symptoms of autonomic dysfunction after treatment of spontaneous intracranial hypotension and orthostatic intolerance was confirmed with head-up tilt table test. Conclusions: Patients with spontaneous intracranial hypotension failing to improve following epidural blood patching should be evaluated for orthostatic intolerance.

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Demographic and clinical features of pediatric patients with orthostatic intolerance and an abnormal head-up tilt table test; A retrospective descriptive study

Pediatrics & Neonatology ◽

10.1016/j.pedneo.2019.06.012 ◽

2020 ◽

Vol 61 (1) ◽

pp. 68-74

Author(s):

Anand Gourishankar ◽

Mathew D. Belton ◽

S. Shahrukh Hashmi ◽

Ian J. Butler ◽

Jeremy E. Lankford ◽

...

Keyword(s):

Clinical Features ◽

Pediatric Patients ◽

Orthostatic Intolerance ◽

Descriptive Study ◽

Tilt Table Test ◽

Tilt Table ◽

Abnormal Head ◽

Head Up Tilt

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Cerebral Perfusion Monitoring Using Near-Infrared Spectroscopy During Head-Up Tilt Table Test in Patients With Orthostatic Intolerance

Frontiers in Human Neuroscience ◽

10.3389/fnhum.2019.00055 ◽

2019 ◽

Vol 13 ◽

Cited By ~ 4

Author(s):

Yoo Hwan Kim ◽

Seung-ho Paik ◽

Zephaniah Phillips V ◽

Nam-Joon Jeon ◽

Byung-Jo Kim ◽

...

Keyword(s):

Infrared Spectroscopy ◽

Near Infrared Spectroscopy ◽

Cerebral Perfusion ◽

Near Infrared ◽

Orthostatic Intolerance ◽

Tilt Table Test ◽

Tilt Table ◽

Head Up Tilt

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A clinical manifestation-based prediction of haemodynamic patterns of orthostatic intolerance in children: a multi-centre study

Cardiology in the Young ◽

10.1017/s1047951113000929 ◽

2013 ◽

Vol 24 (4) ◽

pp. 649-653 ◽

Cited By ~ 6

Author(s):

Li Chen ◽

Xueying Li ◽

Ochs Todd ◽

Cheng Wang ◽

Hongfang Jin ◽

...

Keyword(s):

Clinical Study ◽

Diagnostic Accuracy ◽

Clinical Manifestation ◽

Orthostatic Intolerance ◽

Vasovagal Syncope ◽

Postural Orthostatic Tachycardia Syndrome ◽

Tilt Table Test ◽

Tilt Table ◽

Multi Centre Study ◽

Head Up Tilt

AbstractObjective: At present, the haemodynamic diagnosis of orthostatic intolerance is based mainly on the head-up tilt table test, which is sometimes risky for patients. Thus, it is important to find objective and safe methods to differentiate haemodynamic patterns of orthostatic intolerance cases. Methods: In all, 629 children with orthostatic intolerance, either vasovagal syncope or postural orthostatic tachycardia syndrome, were included in the multi-centre clinical study. We analysed the association between the clinical manifestation and haemodynamic patterns of the patients. Results: Syncope after motion with a prodrome of chest distress or palpitations and the concomitant symptom(s) after a syncopal attack, with debilitation, dizziness or headache, were the most important variables in predicting the diagnosis of vasovagal syncope. The overall diagnostic accuracy was 71.5%. Conclusion: Complaint of syncope after motion with prodromal chest distress or palpitation and the concomitant symptom after a syncopal attack, with subsequent debilitation, dizziness or headache, were the most important variables in the diagnosis of vasovagal syncope in children with orthostatic intolerance.

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Correlation of head-up tilt table test time interval with risk of recurrent syncope

Indian Heart Journal ◽

10.1016/j.ihj.2015.10.189 ◽

2015 ◽

Vol 67 ◽

pp. S77

Author(s):

R.K. Gokhroo ◽

Kumari Priti ◽

A. Avinash ◽

Bhanwar Lal Ranwa ◽

Kamal Kishor ◽

...

Keyword(s):

Test Time ◽

Time Interval ◽

Tilt Table Test ◽

Tilt Table ◽

Head Up Tilt

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Repolarization disparity as a predictor of response to Head Up Tilt‐table Test in pediatric syncope

Pacing and Clinical Electrophysiology ◽

10.1111/pace.14305 ◽

2021 ◽

Author(s):

Mohammad Reza Khalilian ◽

Abdolrahim Ghasemi ◽

Narges Khazaei ◽

Sara Khoshkhou ◽

Elham Mahmoudi

Keyword(s):

Tilt Table Test ◽

Tilt Table ◽

Head Up Tilt

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Cardiovascular Autonomic Nervous System Dysfunction in Patients with Systemic Lupus Erythematosus

QJM ◽

10.1093/qjmed/hcaa052.064 ◽

2020 ◽

Vol 113 (Supplement_1) ◽

Author(s):

D A Hussein ◽

A B E Abdelaziz ◽

A M Abdelmoteleb ◽

M A Elserwy ◽

S G Arafa

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Autonomic Neuropathy ◽

Autonomic Dysfunction ◽

Lupus Erythematosus ◽

Activity Index ◽

Tilt Table Test ◽

Systemic Lupus ◽

Tilt Table ◽

Increased Risk

Abstract Background involvement of central and peripheral nervous systems are well-documented in SLE but literature on prevalence and pattern of autonomic neuropathy are few. Women with SLE have been found to have 5-8 fold increased risk of coronary heart disease. Autonomic dysfunction in the presence of structural cardiovascular disease is associated with adverse prognosis. Aim of the Work the purpose of this study is to evaluate the cardiovascular autonomic function in patients with Systemic lupus erythematosus, and to correlate it with SLE clinical features. Patients and Methods we conducted our study on one-hundred Egyptian SLE patients diagnosed according to SLICC classification criteria for SLE recruited from rheumatology outpatient clinic and inpatient department at Ain Shams University Hospitals. All the studied patients were subjected to history taking, clinical examination, and laboratory and radiological investigations related to SLE diagnosis and activity. SLE disease activity was assessed by SLE disease activity index-2000 (SLEDAI-2K).Tilt table test was used to assess for presence of cardiovascular autonomic neuropathy. Results we found that fifty (50%) of our SLE patients had symptoms suggestive for autonomic dysfunction, including orthostatic hypotension, palpitation and or Raynaud’s phenomenon. Regarding the result of tilt table test, sixty eight (68%) of the studied SLE patients were found to have positive tilt table responses. By doing the uni-variate analysis for predictors of SLE patients with positive tilt table test, we found that vasculitis, proteinuria, DNA, low TLC and complement levels, high SLEDAI, ESR and urinary P/C all were predictors for positivity of tilt table testing. Conclusion The prevalence of autonomic dysfunction was high among SLE patients.

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The Differences in the Diagnostic Profile in Children with Vasovagal Syncope between the Result of Head-Up Tilt Table Test

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph17124524 ◽

2020 ◽

Vol 17 (12) ◽

pp. 4524

Author(s):

Ewelina Kolarczyk ◽

Lesław Szydłowski ◽

Agnieszka Skierska ◽

Grażyna Markiewicz-Łoskot

Keyword(s):

Medical Records ◽

Vasovagal Syncope ◽

Speech Disorders ◽

Tilt Table Test ◽

Tilt Table ◽

Prodromal Symptoms ◽

Close Relationship ◽

Head Up Tilt ◽

Diagnostic Profile ◽

Positive Results

(1) Background: The features characterizing vasovagal syncope (VVS) are an important factor in the correct evaluation of diagnostic risk stratification in children and adolescents. The aim of the study was to determine the value of identifying the clinical characteristics in children with VVS. (2) Methods: We made a retrospective analysis of the medical records of 109 children with diagnosed VVS. We investigated the specific characteristics of syncope in children with VVS including the positive VVS (+) and negative VVS (−) result of the Head-Up Tilt Table Test (HUTT). (3) Results: We did not observe significant differences in the prodromal symptoms of VVS with HUTT response. In addition to typical prodromal symptoms, no difference in statistically reported palpitations (35/109 or 32.1%) and chest discomfort (27/109 or 27.7%) were recorded. Fear–pain–stress emotions as circumstances of syncope were more often reported by children with a negative HUTT (p = 0.02). Cramps–contractures (p = 0.016) and speech disorders (p = 0.038) were significantly higher in the group with negative HUTT. (4) Conclusions: There is a close relationship in the diagnostic profile between the negative and positive results of head-up tilt table test in children with vasovagal syncope.

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Postural orthostatic tachycardia syndrome in primary care: diagnosis, treatment and a case of African-American man presenting with POTS

BMJ Case Reports ◽

10.1136/bcr-2019-229824 ◽

2019 ◽

Vol 12 (9) ◽

pp. e229824

Author(s):

Andrew T Del Pozzi ◽

Michael Enechukwu ◽

Svetlana Blitshteyn

Keyword(s):

Heart Rate ◽

Differential Diagnosis ◽

African American ◽

Clinical Features ◽

Orthostatic Intolerance ◽

Treatment Options ◽

Postural Orthostatic Tachycardia Syndrome ◽

Tilt Table Test ◽

Tilt Table ◽

Caucasian Women

Postural orthostatic tachycardia syndrome (POTS) is a heterogeneous autonomic disorder characterised by orthostatic intolerance and a rise in heart rate by at least 30 bpm or an absolute heart rate value of at least 120 bpm within 10 min of standing or during a tilt table test. Overwhelmingly, POTS affects young Caucasian women, which can lead physicians to miss the diagnosis in men or non-white patients. We describe a case of 29-year-old African-American man who developed lightheadedness, generalised weakness, tachycardia and palpitations and was subsequently diagnosed with POTS. We review its clinical features, differential diagnosis, pathophysiology and treatment options. We also emphasise that POTS should be considered as a differential diagnosis in any patient presenting with typical clinical features, who may not be in the usual demographics of the disorder.

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