Treatment Of Overlap Syndrome Research Articles

Overlap syndrome in liver diseases in children: case report and literature review

In children, autoimmune liver diseases can manifest in a variety of forms, including autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and the co-occurrence of AIH and PSC - the “overlap syndrome.” Early recognition of overlap syndrome is important. Diagnosis of sclerosing cholangitis largely depends on the cholangiographic picture showing single or multiple strictures with dilatation of the biliary tract. The problems of etiology, pathogenesis, diagnosis and treatment of overlap syndrome in children are shown. A clinical example of seronegative autoimmune hepatitis and sclerosing cholangitis as part of a cross syndrome in a child is given.

Biliary dyskinesia in children with irritable bowel syndrome: overlap syndrome. The search for a modern approach to the treatment of abdominal pain syndrome

Objective. To optimize therapeutic approaches to the treatment of abdominal pain syndrome in children with overlapping syndrome of biliary dyskinesia + irritable bowel syndrome.Characteristics of children and research methods. The study included 309 children aged from 13 to 17 years. Irritable bowel syndrome was diagnosed in 76 (24.6%) patients in accordance with the Rome IV revision criteria and the results of laboratory and instrumental studies. 45 (59.2%) patients had a variant of the syndrome with constipation, 19 (25%) – a variant with diarrhea, 12 (15.8%) – a mixed variant (alternating constipation with diarrhea). Biliary pain was detected in 58 (76.3%) patients, including 34 (44.7%) patients with constipation, 13 (17.1%) with diarrhea variants and 11 (14.5%) with mixed option. The researchers used two treatment options: trimebutin (Trimedat, ValentaPharm, Russia) in an age dosage for 28 days (main group) and papaverine in an age dosage for 4 weeks (comparison group).Results. The main group demonstrated a more pronounced decrease in the intensity of abdominal pain associated with both irritable bowel syndrome and biliary dyskinesia. Differences between treatment results in the main and control groups are statistically significant (p<0.01). During the treatment period there were no cases of side effects in the main group, the differences in the frequency of side effects between the main group and the comparison group are statistically significant. (p=0.01).Conclusion. Correction of abdominal pain with trimebutin significantly exceeds the effectiveness of standard antispasmodic therapy in the treatment of overlap syndrome of irritable bowel syndrome + biliary dyskinesia. Trimebutin therapy is safe and can be recommended as a fairly effective method of treating children with overlap syndrome.

A review of therapies for the overlap syndrome of obstructive sleep apnea and chronic obstructive pulmonary disease.

Obstructive sleep apnea (OSA) and chronic obstructive pulmonary disease (COPD) are common chronic diseases. These two noncommunicable diseases (NCDs) are prevalent among approximately 10% of the general population. Approximately 1% of the population is affected by the co‐existence of both conditions, known as the overlap syndrome (OS). OS patients suffer from greater degrees of nocturnal oxygen desaturation and cardiovascular consequences than those with either condition in isolation. Besides OS, patients with COPD may suffer from a spectrum of sleep‐related breathing disorders, including hypoventilation and central sleep apnea. The article provides an overview of the pathogenesis, associated risk factors, prevalence, and management of sleep‐related breathing disorders in COPD. It examines respiratory changes during sleep caused by COPD and OSA. It elaborates upon the factors that link the two conditions together to lead to OS. It also discusses the clinical evaluation and diagnosis of these patients. Subsequently, it reviews the pathophysiological basis and the current evidence for three potential therapies: positive airway pressure therapy [including continuous positive airway pressure (CPAP) and bilevel positive airway pressure], oxygen therapy, and pharmacological therapy. It also proposes a phenotypic approach toward the diagnosis and treatment of OS and the entire spectrum of sleep‐related breathing disorders in COPD. It concludes with the current evidence gaps and future areas of research in the management of OS.

Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis.

The treatment of overlap syndromes is guided by small observational studies whose data have never been synthesized in a rigorous, quantitative manner. We conducted a systematic review and meta-analysis to evaluate the efficacy of available treatments for these rare and morbid conditions. We searched the literature for studies comparing ≥2 therapies for autoimmune hepatitis (AIH)-primary biliary cholangitis (PBC), AIH-primary sclerosing cholangitis (PSC), PBC-PSC, AIH-PBC-PSC, autoimmune cholangitis (AIC), or autoimmune sclerosing cholangitis (ASC) with respect to various clinical outcomes, including biochemical improvement and transplant-free survival. A total of 28 studies met the inclusion criteria for AIH-PBC, AIH-PSC, AIC, and ASC. AIH-PBC patients tended to experience more biochemical improvement with ursodeoxycholic acid (UDCA) + [corticosteroids and/or antimetabolites], i.e., “combination therapy”, than with corticosteroids ± azathioprine (RR = 4.00, 95% CI 0.93–17.18). AIH-PBC patients had higher transplant-free survival with combination therapy than with UDCA, but only when studies with follow-up periods ≤90 months were excluded (RR = 6.50, 95% CI 1.47–28.83). Combination therapy may therefore be superior to both UDCA and corticosteroids ± azathioprine for the treatment of AIH-PBC, but additional studies are needed to show this definitively and to elucidate optimal treatments for other overlap syndromes.

Therapeutic Effect Observation of Tiotropium Bromide in the Treatment of Overlap Syndrome

Objective: To study the M receptor blocker on inhalation in patients with overlap syndrome (chronic obstructive pulmonary disease and Obstructive sleep apnea syndrome) curative effect analysis. Methods: 25 patients with overlap syndrome as the experimental group, chronic obstructive pulmonary disease patients (30) as control group, patients with overlap syndrome use inhaled tiotropium powder treat 30 days, to observe the changes of pulmonary function, polysomnography, and other indicators after treatment. Results: Overlap syndrome were treated by tiotropium bromide inhalation powder, has improved the pulmonary function, the sleep apnea index and lowest nocturnal oxygen saturation after treatment. Conclusion: tiotropium bromide has a preferable effective in treatment of overlap syndrome, COPD and OSAHS are interacting with each other.

Research Priorities in Pathophysiology for Sleep-disordered Breathing in Patients with Chronic Obstructive Pulmonary Disease. An Official American Thoracic Society Research Statement.

Obstructive sleep apnea (OSA) and chronic obstructive pulmonary disease (COPD) are common conditions; the co-occurrence of these diseases, called the overlap syndrome (OVS), has been associated with poor health outcomes. The purpose of this Official American Thoracic Society Research Statement is to describe pathophysiology, epidemiology, outcomes, diagnostic metrics, and treatment of OVS, as well as to identify important gaps in knowledge and make recommendations for future research. Clinicians and researchers with expertise in sleep medicine, pulmonary medicine, or both were invited to participate. Topics were divided among the participants according to their interest and expertise. A literature search was conducted; the search was not a formal systematic review. Evidence was considered and supplemented with the panelists' nonsystematic clinical observations. Important knowledge gaps were identified. Recommendations for research to fill existing knowledge gaps were made. The recommendations were formulated by discussion and consensus. Many important questions about OVS exist. This American Thoracic Society Research Statement highlights the types of research that leading clinicians and researchers believe will have the greatest impact on better understanding the spectrum of disease, improving diagnosis, and optimizing therapy.

Overlap syndromes in hepatic autoimmune
 diseases

Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) comprise a group of autoimmune hepatic disorders. Sometimes, overlap syndromes occur, e.g. AIH/PBC or AIH/PSC. The authors consider autoimmune hepatic disorders with reference to pathogenesis, clinical picture, diagnostics, and treatment of overlap syndromes.

Overlap Syndromes in Inflammatory Myopathies

Purpose of Review This review discusses the epidemiology, evaluation and treatment of overlap inflammatory myopathy syndromes. Recent findings Evaluation of overlap myopathy in systemic sclerosis (SSc) has shown that the disease is often heterogeneous with variable presentation, histopathology, and response to therapy. These differences have shown to be important in management, specifically with the response to immunosuppression. Inflammation and necrosis are features suggestive of a positive response, whereas fibrosis and neurogenic atrophy are also common and may be less responsive to therapy. Summary Inflammatory myopathy associated with overlap connective tissue disease is often associated with greater morbidity, and there are few studies to guide treatment. Treatment is often tailored to the patient based on severity of muscular and concomitant extramuscular disease. While further research is needed to better understand treatment of overlap syndromes, the absence of consensus criteria remains a limiting factor.

Management of Overlap Syndrome between Functional Dyspepsia and Irritable Bowel Syndrome by Western and Traditional Chinese Medicine

The overlap syndrome of functional dyspepsia and irritable bowel syndrome (FD-IBS) is very common and difficult to treat. There are many risk factors of FD-IBS. Mental illness of FD-IBS patients is more serious. Functional dyspepsia and irritable bowel syndrome have some similarities in the aspects of pathophysiology, pathogenesis, and treatment. We should pay attention to two aspects of the treatment of overlap syndrome, one is simplifying medications, the other is using gastrointestinal motility drug with bidirectional regulative function when necessary. Traditional Chinese medicine in this respect shows some advantages. This review addresses the epidemiology, risk factors, clinical features, pathogenesis and management of FD-IBS.

Overlap Syndromes

Background: Some patients present with features of both primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC) on the one hand and autoimmune hepatitis (AIH) on the other hand, either simultaneously or consecutively. The term ‘overlap syndrome (OS)' is used to describe these settings, but lack of universal agreement on what precisely constitutes an OS has generated considerable confusion. The low prevalence of OS (roughly 10% of PBC or PSC) has made it impracticable to perform randomized controlled trials. It remains unclear whether this syndrome forms a distinct entity or is a variant of PBC, PSC or AIH. Key Messages: Moderate to severe interface hepatitis is a fundamental component and histology is vital in evaluating patients with overlap presentation. Use of the International Autoimmune Hepatitis Group criteria for the diagnosis of OS is not recommended. For PBC-AIH OS, EASL has provided diagnostic criteria and, in most cases, it is possible to define one primary disorder (‘dominant' disease), usually PBC. Patients with OS seem to have a more severe disease compared to conventional PBC. PSC-AIH OS is assumed to exist in a considerable part of mainly young patients with autoimmune liver disease and long-term progression towards cirrhosis seems to occur in the majority of cases. In children, the hepatitic feature can be very dominant, and up to 50% of pediatric AIH have cholangiographic abnormalities suggestive of PSC (autoimmune sclerosing cholangitis). Treatment of OS is empiric and includes ursodeoxycholic acid for the cholestatic component (depending on local policy for PSC) and immunosuppressive agents for the hepatitic component, either simultaneously or sequentially. The dominant clinical feature should be treated first and therapy adjusted according to the response. Conclusions: OS is not uncommon but should not be over-diagnosed in order not to expose unnecessarily PBC or PSC patients to the risk of steroid side effects. Therapy has to be individualized and not be static.

Efficacy of auto-trilevel positive airway ventilation in hypercapnic patients with overlap syndrome

To observe the efficacy of auto-trilevel positive airway pressure (Auto-trilevel PAP) ventilation in overlap syndrome (OS) patients with hypercapnia. From October 2012 to May 2014, 21 OS patients with a PaCO2> 45 mmHg (1 mmHg = 0.133 kPa) and coexisting stable chronic obstructive pulmonary diseases (COPD) and moderate-to-severe OSAS were recruited. Three different positive airway pressure (PAP) modes of ventilators (SOMNOvent auto-S, Weinmann Inc, Germany) were employed for 8 hours per night with each mode at each night and two nights' interval without any treatment among different modes. Under mode one, the expiratory positive airway pressure (EPAP) issued by bilevel positive airway pressure (BiPAP) was titrated as the minimal positive pressure for a disappearance of snoring. The same inspiratory positive airway pressure (IPAP) titrated by end tidal CO2 levels under mode 1 was used under modes 2 and 3 as well. However, the EPAP issued by BiPAP under mode 2 was 3 cmH2O (1 cmH2O = 0.098 kPa) higher than that under mode 1. Under mode 3 with autotrilevel PAP, the beginning of EPAP was set the same as that under mode 1 while the end of EPAP (EEPAP) was automatically adjusted based on upper airway patency condition. Comparisons were made for parameters before and after treatment as well as among different ventilation modes. The following parameters were compared such as nocturnal apnea hypopnea index (AHI), minimal SpO2 (miniSpO2), arousal index, sleep efficiency, morning PaCO2 and Epworth sleepiness score (ESS). Compared with the parameters pre-therapy, modes 1-3 showed a significant decrease in nocturnal AHI (6.3 ± 1.3), (3.1 ± 1.0), (3.6 ± 0.6) vs (38.6 ± 11.3) events/h), arousal index ((7.0 ± 1.1), (5.1 ± 0.9), (4.2 ± 1.7) vs (27.5 ± 5.4) events/h), morning PaCO2 (42.4 ± 3.8), (47.9 ± 2.6) and (43.2 ± 3.3) vs (57.3 ± 4.3) mmHg and daytime ESS (7.2 ± 1.3, 7.4 ± 1.3 and 5.3 ± 1.5 vs 11.4 ± 2.7), but a significant increase in nocturnal miniSpO2 (82.3 ± 5.4), (89.6 ± 3.9) and (90.3 ± 3.2) vs (62.4 ± 11.3) mmHg and sleep efficiency caused (71.3 ± 5.2)%, (79.4 ± 4.3)% and (83.2 ± 4.4)% vs (59.8 ± 6.3)% (all P < 0.01). Comparison of 3 modes demonstrated that, with the same IPAP, mode 3 resulted in the lowest arousal index, daytime ESS and the highest sleep efficiency. Comparison of modes 1 and 2 revealed a statistically lower AHI but higher miniSpO2 and morning PaCO2 under mode 2 (all P < 0.01). Compared with mode 1, mode 3 showed a lower AHI, higher miniSpO2 (all P < 0.01), but there was no significant difference in PaCO2 at the end of therapy. Compared with mode 2, mode 3 showed a significant lower PaCO2 (P < 0.01), but there was no significant difference in AHI and miniSpO2. Auto-trilevel PAP ventilation is superior to fixed BiPAP ventilation in the treatment of hypercapnic OS since this novel PAP mode can achieve a higher efficacy in simultaneous removal of residual apnea hypopnea events and correction of hypercapnia and yield a higher sleep quality and lower daytime sleepiness.

Obstructive Sleep Apnea in Patients with Chronic Obstructive Pulmonary Disease

Obstructive sleep apnea (OSA) and chronic obstructive pulmonary disease (COPD) are common disorders in clinical practice and are associated with significant cardiovascular morbidity. The simultaneous occurrence of OSA and COPD happens frequently and is referred to as an overlap syndrome. These patients often have very poor quality sleep and more nocturnal hypoxemia. This combination may increase the severity of metabolic complications and cardiovascular disease, and these patients have increased mortality when compared to patients with either COPD or OSA alone. The treatment of overlap syndrome should focus on both coexisting diseases and includes continuous positive airway pressure, oxygen supplementation, and medications for chronic lung disease.

Obstructive Sleep Apnea in Chronic Obstructive Pulmonary Disease: The Overlap Syndrome

Respiratory irregularities normally occur during sleep. In populations afflicted with obstructive sleep apnea (OSA) or chronic pulmonary disease (COPD), these breathing irregularities become more pronounced and subsequently lead to adverse effects on health. Overlap syndrome (OS) is defined as the co-existence of COPD and OSA in the same patient. Patients with overlap syndrome have a worse prognosis compared to those with either COPD or OSA alone. The purpose of this report is to describe the pathophysiology of COPD and OSA, as each disease process contributes uniquely to the development of OS. We also describe the epidemiology, clinical presentation, diagnosis, prognosis, and treatment of OS. The diagnosis of OS is challenging due to variable clinical presentations. Symptoms such as fatigue, are subtle and non-specific; therefore, clinicians should have a high index of suspicion for diagnosis. Diagnostic tools include pulmonary function tests to confirm chronic obstruction as well as polysomnography to evaluate breathing patterns during sleep. Presence of apneas and hypopneas associated with concomitant arousals and hypoxia, contribute to the diagnosis of sleep apnea in COPD patients. Clinicians should treat overlap syndrome by optimizing bronchodilator therapy for COPD and administering continuous positive airway pressure with or without supplemental oxygen during sleep. Prompt diagnosis and treatment benefits the patient not only by providing practical relief from symptoms such as daytime fatigue; more importantly, treatment of OS significantly impacts health by helping to reduce cardiovascular complications.

Autoimmune liver disease - are there spectra that we do not know?

Autoimmune liver diseases (AILDs) are common leading causes for liver cirrhosis and terminal stage of liver disease. They have variable prevalence among patients with liver disease and have two major clinical and biochemical presentations. Autoimmune hepatitis (AIH) is the typical example of hepatocellular AILD, but it can also be presented under a cholestatic pattern. AIH has a scoring diagnostic system and respond in most cases to the treatment with prednisolone and azathioprine. Primary biliary cirrhosis (PBC) is the second most common AILD, with a cholestatic presentation and characterized by positive antimitochondrial antibody (AMA). It has an excellent response and long term outcome with the administration of ursodeoxycholic acid (UDCA). Another AILD that is thought to be a variant of PBC is the autoimmune cholangitis, being a disease that has biochemical and histological features similar to PBC; but the AMA is negative. Primary sclerosing cholangitis (PSC) is a rare entity of AILD that has a cholestatic presentation and respond poorly to the treatment, with the ultimate progression to advance liver cirrhosis in most patients. Other forms of AILD include the overlap syndromes (OS), which are diseases with mixed immunological and histological patterns of two AILD; the most commonly recognized one is AIH-PBC overlap (AIH-PSC overlap is less common). The treatment of OS involves the trial of UDCA and different immunosuppressants. Here we present three case reports of unusual forms of chronic liver diseases that most likely represent AILD. The first two patients had a cholestatic picture, whereas the third one had a hepatocellular picture at presentation. We discussed their biochemical, immunological and histological features as well as their response to treatment and their outcomes. Then, we compared them with other forms of AILD.

Regulation of expiratory airway pressure of noninvasive ventilation for the treatment of overlap syndrome with respiratory failure

Objective To evaluate the clinical effect of regulating expiratory airway pressure of noninvasive ventilation(NIV)to treat overlap syndrome(OS)with respiratory failure by pressure titration in monitor during sleep.Method Individuals who met the following criteria were selected for the study:(1)OS with respiratory failure confiemed by polysomnogram(PSC)and pulmonary function tests;(2)ability to tolerate PSG tests all night;(3)lucid consciousness and spontaneous breathing upon awaken and(4)ability to tolerate NIV.The criteria of exclusion were:(1)frequent expectoration of blood or hemoptysis;(2)spontaneous pneumothorax;(3)severe prosopo-trauma or facial ulcer and(4)large amount of secretion in the airway.From August 2005 to Decentber 2008,27 patients were selected with two patients excluded.The remaining 25 patients treated with noninvasive bi-level positive airway pressure(BiPAP)were randomly assigned to titration group or control group.During sleep,the patients of titration group were put under the optimal expiratory airway pressure[EPAP,(8.8 ± 0.5)cmH2O]which was got from titration by AutoCPAP ventilator and the EPAP decreased immediately to 5 cmH2O when the patients wake up next day.In the patients of control group,the EPAP was maintained at 5 cm H2O round the clock.The degree of apnoea and the hypopnea index(AHI),the results of arterial blood gas analysis(pH,PaO2,PaCO2)in the morning,the lowest pulse oxygen saturation(SPO2LOW)and the arterial blood gas level at 10 am before and after ventilation,duration of taking off ventilator at night,complications of NIV,incidence of air leakage and invasive ventilation after NIV were comparatively analyzed between the two groups.Results Compared with the control group,the titration group showed a notably increase in the value of SPO2LOW[(25.9± 11.3)%vs.(14.9±8.4)%;P＜0.05].The PaO2 level was significantly higher in the titration group(29.4±4.4)mmHg than that in the control group(22.6 ± 2.1)mmHg(P＜0.0001);however,the PaCO2 level in the titration group(16.8 ±7.3)mmHg was not significant different from that in the control group(20.0±6.3)mmHg(P＞0.05).The PaO2 level at 10am in the titration group(28.9 ± 6.7)mmHg was not significant different from that in the control group(31.3 ± 7.7)mmHg(P＞0.05);however,the PaCO2 level in the titration group(23.3 ±5.7)mmHg was higher than that in the control group(18.2 ±5.0)mmHg(P＜0.05).Conclusions Regulating EPAP of NIV to treat overlap syndrome with respiratory failure by pressure titration in monitor during sleep was more effective at improving hypoxemia and carbon dioxide retention than traditional methods without adjusting the EPAP to the optimal level.The optimal didn't increase carbon dioxide retention at night,confirming that the method was safe. Key words: Sleep monitor; Overlap syndrome; Noninvasive ventilation; Respiratory failure; Obstructive sleep apnea syndrome; Chronic obstructive pulmonary diseases

EPOC y síndrome de apnea-hipopnea del sueño

Overlap syndrome refers to the coexistence of COPD and obstructive sleep apneas-hypopneas in the same patient. This syndrome affects 1% of the adult population. There is no particular clinical presentation but patients with overlap syndrome show COPD with disproportionate hypercapnia, hypoxemia and dyspnea. These patients usually report loud snoring, poor quality of sleep and daytime drowsiness. There is also a tendency to rapidly develop pulmonary hypertension. The natural history of the syndrome is largely unknown but some preliminary results from the Zaragoza Sleep Cohort Study indicate a high probability of long-term cardiovascular mortality in patients with overlap syndrome not treated with non-invasive ventilation. Treatment of the syndrome is nonspecific. The treatment of overlap syndrome is specific to each of the two components and consequently the management of COPD should be individually tailored and the severity of obstructive sleep apnea-hypopnea syndrome and response to non-invasive ventilation should be evaluated.