ABSTRACT
Background: Shrinking lung syndrome (SLS) is an uncommon complication of systemic lupus erythematosus (SLE) that has also been seen in other autoimmune diseases and is linked with a high risk of acute or chronic respiratory failure. Alveolar hypoventilation in the presence of obesity-hypoventilation syndrome, systemic lupus erythematosus (SLE), and myasthenia gravis (MG) is uncommon and poses a diagnostic and therapeutic challenge. Case report: We reported a 33-year-old female patient from Saudi Arabia who suffered from obesity, bronchial asthma, newly diagnosed essential hypertension, type 2 diabetes mellitus, with recurrent acute alveolar hypoventilation, secondary to obesity hypoventilation syndrome and mixed autoimmune disease (systemic lupus erythematosus and myasthenia gravis), based on the correct constellation of clinical findings and laboratory evidence. Conclusion: The interesting aspect of this case report: is the presentation of the overlap of obesity hypoventilation syndrome and shrinking lung syndrome due to systemic lupus erythematosus with generalized and respiratory muscle dysfunction due to myasthenia gravis with good outcomes after therapy.
ABSTRACT
Background and Aim: Behçet disease (BD) is a rare chronic relapsing-remitting inflammatory systemic vasculitis. BD patients were reported to have marked acceleration of subclinical atherosclerosis (SCA). Endocan is a soluble proteoglycan mainly secreted by the activated endothelium. The present study aimed to assess the relation between serum endocan levels and SCA in BD patients. Subjects and Methods: The study included 40 adult BD patients in addition to twenty age- and sex-matched healthy controls. BD was diagnosed according to International Study Group criteria. Upon recruitment, all participants were subjected to careful history taking and thorough clinical examination. BD activity was assessed using Behçet Syndrome Activity Score. Measurement of serum endocan was performed using quantitative double-antibody sandwich ELISA kit. CIMT measurement was done using B-mode ultrasound. Results: Comparison between patients and controls regarding serum endocan levels revealed significantly higher endocan levels in BD patients [median (IQR): 155.0 (69.3-610.0) versus 73.8 (51.9-94.6)]. Using ultrasound assessment, SCA was found in 14 BD patients (35.0%). Comparison between patients with SCA and patients without regarding the clinical and laboratory data revealed that the former group had significantly higher CRP [median (IQR): 36.5 (26.8-43.5) versus 21.0 (11.8-26.8) mg/dL, p < 0.001] and endocan [median (IQR): 622.0 (107.4-974.8) versus 104.5 (64.0-342.0) mg/dL, p = 0.004] levels. Logistic regression analysis recognized endocan [OR (95% CI): 1.0 (1.0-1.012), p0.035] levels as significant predictor of SCA in multivariate analysis. Conclusion: The present study identified the clinical value of serum endocan levels as a possible early marker of vascular involvement in BD patients.
