Lung Transplantation under a Janus Kinase Inhibitor in Three Patients with SAVI Syndrome.

Stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) is a very rare autoinflammatory disease related to STING1 mutation. SAVI is mainly characterized by fever attacks and skin and respiratory manifestations such as interstitial lung disease or alveolar hemorrhage. Respiratory involvement occurs in 80% of cases and might progress to severe lung fibrosis and require lung transplantation (LT). Three patients with SAVI who underwent LT have been reported to date. Two of the three patients died months or years after LT due to multiple organ failure or sepsis. However, the diagnosis of SAVI was made after LT, thus preventing the use of targeted therapy, such as the Janus kinase 1 and 2 inhibitor (JAK1/2i) ruxolitinib, which might be beneficial for the respiratory status of these patients. We aimed to report our experience in managing three patients who were followed in three large lung transplantation centers in France and who benefited from ruxolitinib before undergoing LT. We describe posttransplant complications that occurred as well as outcomes.

Psychological status in Moroccan patients with ankylosing spondylitis and its relationships with disease parameters and quality of life.

BACKGROUND: The evaluation of emotional status is an important parameter in management of rheumatic diseases. There are few studies evaluating the psychological status in ankylosing spondylitis (AS) and its relationships with disease parameters and quality of life. OBJECTIVE: In this study, we evaluated the psychological status in Moroccan patients with AS and its relationships with the activity of the disease, the functional status, and the quality of life. PATIENTS: One hundred ten patients were included in this cross-sectional study according to the modified New York criteria for AS. Psychological status was assessed by the Hospital Anxiety and Depression Scale (HADS) including the depression and anxiety subscales. The quality of life was evaluated by the Short Form 36. RESULTS: Depression was found in 55.5% and anxiety in 60% among our patients. The HADS depression and anxiety subscales were significantly correlated with clinical parameters and with worsening in all domains of the Short Form 36.Multivariate logistic regression analysis revealed that role limitations due to emotional problems, vitality, and general health perception were independent risk factors of anxiety. The Bath Ankylosing Spondylitis Functional Index, vitality, and role limitations due to emotional problems were the independent factors that influenced the risk of depression. CONCLUSION: This study suggests that depression and anxiety are frequent in AS. Impaired quality of life and functional disability seemed to be independent risk factors of psychological disorders. Therefore, assessment and management of patients with AS should take into account the evaluation and management of their psychological disorders and improvement of their functional disability.