ABSTRACT
BACKGROUND: Sarcoidosis is an idiopathic systemic granulomatosis whose evolution is self-limiting in most cases. However, it can progress to organ damage that menaces the vital or functional prognosis of patients. Sarcoidosis itself, but also its comorbidities, can pose a threat to the patient, require rapid initiation of treatment, and justify emergency hospitalization. RESEARCH QUESTION: What are the reasons and prognosis of patients with sarcoidosis hospitalized in emergency? STUDY DESIGN AND METHODS: The objectives of our study were to describe the causes of admission, and to identify predictors of mortality in patients with sarcoidosis hospitalized in emergency. This is a retrospective monocentric study. We included patients hospitalized after a stay in the ED or ICU, or requiring an unscheduled hospitalization after telephone advice or a consultation, between January 1, 2017 and July 7, 2020. RESULTS: We identified 154 patients with sarcoidosis hospitalized in emergency, among which 14 (9%) required the ICU. There were 81 men, with a median age of 55.0 years (interquartile range, 44.0-67.0). Sarcoidosis was inaugural in 20 patients (14%). The primary reason for hospitalization was lower respiratory infections in 32 patients (21%), followed by acute pulmonary exacerbation of sarcoidosis in 17 (11%), suspected cardiac sarcoidosis in 13 (8.4%), and neurosarcoidosis in 12 (7.7%). The median length of stay was 6 days (interquartile range, 3.00-10.0). In-hospital mortality rate was 3.9%. The 2-year transplantation-free survival after hospitalization was 86.8% (95% CI, 81.4-92.5). The factors associated with a worse transplantation-free survival were Charlson Comorbidity Index (hazard ratio [HR], 1.29; 95% CI, 1.04-1.61; P = .021), pulmonary hypertension (HR, 2.53; 95% CI, 1.10-5.83; P = .029), and oxygen therapy during hospitalization (HR, 4.18; 95% CI, 1.55-11.29; P = .005). INTERPRETATION: The overall mortality of patients with sarcoidosis hospitalized in emergency is high. The presence of comorbidities and the severity of respiratory failure, as reflected by oxygen requirement, are important prognostic determinants.
ABSTRACT
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding. A noninvasive diagnostic approach, including oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest, is used to support a diagnosis of PVOD. PVOD prognosis is worse than other forms of PAH. There is no evidence-based medical therapy for PVOD and life-threatening pulmonary edema may occur following PAH targeted therapy in PVOD. Lung transplantation remains the preferred definitive therapy for eligible patients.
Subject(s)
Pulmonary Veno-Occlusive Disease , Animals , Diagnostic Imaging/methods , Disease Models, Animal , Humans , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/therapy , Rare Diseases/diagnosis , Rare Diseases/epidemiology , Rare Diseases/therapy , Respiratory Function Tests/methods , Risk FactorsABSTRACT
INTRODUCTION: Among patients with non-small-cell lung cancer, coexistence of EGFR mutation and ALK rearrangement is rare. We describe the clinical features of two patients with this double anomaly. CASE REPORTS: A 62-year-old Caucasian non-smoking woman was diagnosed with cT4N0M0 lung adenocarcinoma. Initial biopsy showed EGFR mutation and ALK rearrangement. She received cisplatin-gemcitabine, followed by 17 months of gemcitabine. Owing to progression, she received erlotinib for 14 months, then paclitaxel for 6 months and finally crizotinib. A partial response was achieved and maintained for 24 months. A 45-year-old Caucasian woman, light smoker, was diagnosed with cT2N3M0 lung adenocarcinoma. Only EGFR mutation was found on initial analysis. She underwent treatment with cisplatin-gemcitabine and thoracic radiotherapy. Progression occurred after 8 months and afatinbib was started. Eight months later, progression was observed with a neoplasic pleural effusion in which tumor cells expressing ALK rearrangement were found. A new FISH analysis was performed on the initial tumor but did not find this rearrangement. Despite a third line of crizotinib, the patient died one month later. DISCUSSION: The literature shows 45 other cases of these two abnormalities, observed either from the start or during follow-up. EGFR's TKI were almost always given before ALK's TKI. CONCLUSIONS: Therapeutic strategy needs to be clarified in cases of double alteration. With regard to the second patient, appearance of ALK rearrangement may constitute a resistance mechanism to EGFR's TKI.