Background: Some hypersensitivity pneumonitis (HP) patients exhibit autoimmune features (HPAF). This study compared outcomes of HPAF and HP without autoimmune features, focusing on progressive pulmonary fibrosis (PPF) and response to immunosuppression. Methods: A retrospective cohort study included HP patients from a single center. HPAF was defined as HP overlapping with autoimmune disease or presenting autoimmune markers/symptoms not fulfilling connective tissue disease criteria. A control HP group without autoimmune features was randomly selected. Demographics, autoimmune profiles, and outcomes over two years were analyzed. Results: 103 patients were included (52 HPAF; 51 HP). In HPAF, the most common autoimmune diseases were rheumatoid arthritis (9.6%), while 57.7% had isolated autoimmune serology. Groups showed no baseline differences in demographics, exposures, smoking, or lung function. Fibrotic disease on high-resolution CT at diagnosis was less frequent in HPAF (71.2% vs. 88.2%; p = 0.031). At two-year follow-up, survival, transplantation, and PPF prevalence were similar. HPAF patients received immunosuppression less often (69.2% vs. 86.3%; p = 0.038). Among patients under immunosuppression, PPF was significantly lower in HPAF group (8.6% vs. 29.5%; p = 0.021). Conclusions: Within two years post-diagnosis, HPAF and HP had comparable overall outcomes. However, under immunosuppression, HPAF patients had significantly lower odds of developing PPF (adjusted OR 0.08; 95% CI 0.008–0.816; p = 0.033) compared to HP patients, suggesting a more favorable treatment response.

Brown-Vialetto-Van Laere syndrome (BVVLS) is an extremely rare genetic neurological disorder caused by riboflavin transport deficiency, an autosomal recessive condition mostly associated with mutations in the SLC52A2 and SLC52A3 genes. It follows a progressive course, typically characterized by sensorineural deafness, facial weakness, ponto-bulbar palsy, ataxia, and peripheral sensory-motor neuropathy. This disease is often associated with childhood mortality if left untreated. We report the case of a 68-year-old woman who first noticed a mild hearing loss at the age of 12. This was followed by a slowly progressive onset of bilateral facial paresis, dysarthro-dysphonia, stridor, and tongue atrophy with fasciculations. At 63 years of age, genetic testing revealed a single heterozygous variant in the SLC52A3 gene. Although typically autosomal recessive, some individuals with classic symptoms and even response to riboflavin therapy have been found to carry only a single mutation in either the SLC52A2 or SLC52A3 gene. Therefore, given the compatible clinical presentation, a diagnosis of BVVLS was considered after discussion with a center of expertise. Consequently, 10 mg/kg/day of riboflavin supplementation was prescribed for three years, but no significant clinical improvement was observed. Currently, at age 68, the patient is on nocturnal non-invasive mechanical ventilation (NIV) and uses assisted airway clearance techniques, including air-stacking maneuvers and mechanical insufflation-exsufflation on demand, due to respiratory compromise secondary to diaphragmatic weakness and vocal cord paralysis. This unique presentation of slowly progressive symptoms and long survival may be related to the single heterozygous SLC52A3 variant found. Respiratory care in BVVLS is currently adapted from other neuromuscular disorders with stronger evidence bases. This case highlights the critical role of pulmonology in BVVLS care, including clinical and functional monitoring, early initiation of NIV, and the implementation of airway clearance techniques.

The co-occurrence of tuberculosis (TB) and diabetes mellitus (DM) presents a growing public global health concern. Managing DM during anti-TB therapy is challenging due to potential drug-drug interactions, especially with rifamycin (RIF). Assess the effects of anti-TB drugs on the pharmacokinetics and pharmacodynamics of novel antidiabetic agents, including DPP-4 inhibitors (DPP4i), SGLT-2 inhibitors (SGLT2i) and GLP-1 receptor agonists (GLP-1a). A PRISMA-ScR-based scoping review was conducted among four databases. Ten studies involving 307 participants were included. RIF significantly reduced the plasma exposure of DPP4i (saxagliptin, gemigliptin, evogliptin) and canagliflozin, while other SGLT2i (dapagliflozin, empagliflozin, ertugliflozin) were minimally affected. No direct data was available for GLP-1a. Adverse events were rare in healthy participants but more frequent in elderly patients with poorly controlled DM. Linezolid and dapagliflozin co-administration may lead to severe pancytopenia. RIF co-administration with gemigliptin, evogliptin and canagliflozin requires caution and potential requiring dose adjustments, while saxagliptin, dapagliflozin, ertugliflozin and empagliflozin appear safer alternatives. Haematologic monitoring is recommended when combining linezolid and dapagliflozin. However, current evidence remains limited by small sample sizes, single-dose designs, inclusion of mainly healthy participants, and lack of data on GLP-1a or other anti-TB agents. The limited inclusion of DM patients with TB, restricted to one study with latent TB infection, further reduces generalisability. We developed a clinical decision algorithm to support co-treatment in TB - DM cases, but further dedicated studies are warranted to guide optimal co-treatment.

Dear Editor,According to GINA, severe asthma is defined as uncontrolled asthma, despite therapy adherence with an optimized high dose of inhaled corticosteroid plus long-acting ß2-agonist coupled with management of modifiable factors/comorbidities, that worsens when this treatment is decreased. It affects a significant portion of asthmatic patients and imposes a high risk of exacerbations and mortality, which are associated with significant healthcare costs and psychosocial impact...

Central Sleep Apnea (CSA) is an uncommon sleep breathing disorder which diagnosis is based on sleep-related symptoms plus nocturnal polysomnography (PSG) revealing ≥5 central apneas/hypopneas per hour of sleep, with central events being >50% of respiratory events. Although CSA and Obstructive Sleep Apnea (OSA) might coexist, pure CSA is uncommon. The aim of this work was to describe a CSA case series without OSA criteria (obstructive apnea/hypopneas index [AHI] ≤5/h) followed at a reference tertiary hospital in Portugal. Treatment-emergent CSA was excluded. Eight cases were identified. Their age range was 22-86 years, and 5 were male. Daytime sleepiness and witnessed apneas were frequently reported. According to PSG, CSA was classified as: mild (n=1; central AHI= 13/h), moderate (n=2; central AHI= 17.8-22.1/h) and severe (n=5; central AHI 35.8-45.2/h). Cheyne-Stokes breathing (CSB) was present in 3 cases. The majority was normocapnic, only one patient had daytime hypocapnia. Relevant medical conditions (heart failure, arterial fibrillation and chronic kidney disease) were identified in 5 patients, which included CSB cases. The remaining were classified as primary CSA after extensive etiological study, in the absence of pharmacological contribution or secondary cause. Moderate to severe CSA initiated positive airway pressure (PAP) therapy: continuous PAP (n=2), BiLevel-ST (n=1) and adaptive servo-ventilation (n=4, with LVEF=46-60%). Four cases had optimal PAP adherence and symptom improvement. CSA without OSA at a diagnosis PSG is infrequent in most Sleep Medical Centres and their management can be difficult. The authors described 8 cases reflecting different phenotypes and treatment approaches.