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1.
Clin Respir J ; 18(3): e13740, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38497229

ABSTRACT

Lambert-Eaton myasthenic syndrome (LEMS) is a rare paraneoplastic neurological syndrome of the neuromuscular transmission. The symptoms often progress slowly and can be misdiagnosed in early stage. Seropositive SOX-1 antibodies are support for the diagnosis of LEMS and have high specificity for small cell lung cancer (SCLC). In this paper, we report a case of a 56-year-old man with smoking history who was admitted to hospital with progressive muscle weakness of the proximal legs. LEMS was diagnosed by repetitive nerve stimulation (RNS) testing and seropositive SOX-1 antibodies. Primary screening with chest computed tomography (CT) and integrated PET/CT did not reveal any tumor. After continuous follow-up, SCLC was found by chest CT and confirmed with pathological examination 10 months after the diagnosis of LEMS. Long-term follow-up and screening for occult SCLC in LEMS patients with positive SOX-1 antibodies are very important.


Subject(s)
Lambert-Eaton Myasthenic Syndrome , Lung Neoplasms , Small Cell Lung Carcinoma , Male , Humans , Middle Aged , Small Cell Lung Carcinoma/complications , Small Cell Lung Carcinoma/diagnosis , Lung Neoplasms/complications , Lung Neoplasms/diagnosis , Lambert-Eaton Myasthenic Syndrome/complications , Lambert-Eaton Myasthenic Syndrome/diagnosis , Positron Emission Tomography Computed Tomography , Autoantibodies
2.
Am J Case Rep ; 25: e942727, 2024 Feb 11.
Article in English | MEDLINE | ID: mdl-38341610

ABSTRACT

BACKGROUND Managing IgG4-related disease (IgG4-RD) in the context of vascular complications, such as aneurysms, poses significant challenges, particularly when considering surgical intervention options. The risk of rupture and infection in patients on long-term glucocorticoid therapy complicates treatment decisions. CASE REPORT A 63-year-old woman with a history of IgG4-RD presented with a ruptured right iliac artery aneurysm. She was on long-term oral glucocorticoid therapy. Initial emergency endovascular stent graft implantation was followed by embolization for suspected arterial bleeding and subsequent Salmonella bacteremia. Repeated hospitalizations involved stent graft removal and surgical repair due to persistent infection. Over 2 years, the patient required multiple pelvic drainages and long-term antibiotic and prednisolone therapy, yet her quality of life remained compromised. CONCLUSIONS Our case highlights the unique challenges and considerations in the treatment of IgG4-related aneurysms. Patients with IgG4-RD who are on long-term oral glucocorticoids have an inherent risk of aneurysm rupture. We believe regular follow-ups to monitor the progression of the aorta and iliac arteries into aneurysms are essential. For patients who have developed aneurysms, it is advisable to reduce the dosage of glucocorticoids or even consider surgical treatment as soon as possible. As for the choice of surgical method, there is no consensus yet. While endovascular treatment is less invasive and quicker, it can increase the risk of rupture and bleeding. Open surgery might be a better option. More data are needed to make a definitive judgment.


Subject(s)
Aneurysm, Ruptured , Iliac Aneurysm , Immunoglobulin G4-Related Disease , Female , Humans , Middle Aged , Aneurysm, Ruptured/complications , Aneurysm, Ruptured/surgery , Glucocorticoids/adverse effects , Iliac Aneurysm/complications , Iliac Aneurysm/surgery , Iliac Artery/surgery , Immunoglobulin G4-Related Disease/complications , Immunoglobulin G4-Related Disease/surgery , Quality of Life , Stents , Treatment Outcome
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