Presence of certain autoimmune disorders should increase the suspicion of HCL in an appropriate clinico-laboratory context. Conversely, the diagnosis of HCL should prompt early recognition of certain autoimmune disorders if clinical suspicion exists. While some of these autoimmune diseases are thought to be secondary to the dysfunctional immune response associated with underlying malignant process, others could be primary and might even play a role in the HCL pathogenesis. The autoimmune complications can pose important clinical problems for the HCL patients. Therefore, a catalogue of these problems is important for alerting physicians to watch for them and diagnose them promptly.
Purpose of Review To discuss the current evidence regarding the association and mechanistic interaction between asthma and obstructive sleep apnea (OSA). Recent Findings The co-existence of OSA is highly prevalent in asthmatics and significantly associated with increased severity, decreased control, more frequent exacerbations, and hospitalizations despite medical management. Pre-existing asthma may also be a risk factor for new onset OSA. Rhinitis, obesity, and gastro-esophageal reflux are risk factors in both conditions. The obese asthmatic with OSA may present a unique phenotype. Positive airway pressure in severe asthma improves outcomes. Summary Pathophysiologic mechanisms and co-morbidities overlap between OSA and asthma, but the exact link has yet to be confirmed. Screening for OSA is recommended in those with severe asthma. Further investigations are needed to delineate the cellular processes with therapeutic targets. Similarly, prospective investigations are needed to evaluate the longitudinal relationship in pre-existing asthma and the development of OSA.
Introduction Ehlers-Danlos syndrome (EDS) is a genetically inherited connective tissue disorder which has a high prevalence of sleep conditions, including obstructive sleep apnea, insomnia, fatigue, and hypersomnia., Chronic fatigue is an important factor in the impaired quality of life in patients with EDS. Successful treatment of fatigue and hypersomnia with traditional wake-promoting medications and stimulants is limited, due to the high prevalence of postural tachycardia, orthostatic intolerance, and other cardiac conditions in these patients. We present a case of EDS with underlying cardiac comorbidities, hypersomnia and fatigue who had significant improvement in excessive daytime sleepiness after treatment with flumazenil. Report of Case A 19 yoF with PMH of Ehlers-Danlos syndrome, postural tachycardia syndrome (POTS), autonomic instability and well-controlled depression presented with symptoms of fatigue and excessive daytime sleepiness (ESS of 17/24) despite obtaining 10-15 hours of sleep each day. Polysomnogram followed by MSLT was notable for borderline excessive sleepiness without other abnormalities (PSG: AHI 3/hr, SpO2 nadir 92%; MSLT: 0 SOREMS, mean sleep latency 10 minutes). Prior autonomic and cardiac work-up revealed POTS (maximum HR 180 bpm), orthostatic intolerance and aortic root dilatation. Physical exam and previous laboratory work up for fatigue were unremarkable. A trial of flumazenil 6 mg lozenge every 4-6 hours, as needed for sleepiness, was initiated. On her subsequent visit 6 months later, patient reported 50% improvement in symptoms of fatigue and sleepiness, with decrease in ESS from 17 to 5. No adverse effects to flumazenil were reported. Conclusion Flumazenil is a gamma-aminobutyric acid (GABA)-A receptor antagonist that has been previously documented to provide sustained clinical benefit in treatment-refractory hypersomnolence. This case report highlights a successful alternative treatment option for hypersomnolence in patients with EDS and cardiac comorbidities, in which traditional wake-promoting agents and stimulants may be contraindicated.
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