The diagnosis of sarcoidosis relies on clinical and radiological presentation, evidence of non-caseating granulomas in histopathology and exclusion of alternative causes of granulomatous inflammation. Currently, a proper diagnosis, with a high level of confidence, is considered as key to the appropriate diagnosis and management of the disease. In this sense, this review aims to provide a brief overview on the role of bronchoscopy in the diagnosis of thoracic sarcoidosis, incorporating newer techniques to establish, including endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), transesophageal ultrasound-guided needle aspiration with the use of an echo bronchoscope (EUS-B-FNA) and transbronchial lung cryobiopsy (TBLC). Most of the literature reports the diagnostic superiority of endosonographic techniques, such as EBUS-TBNA alone or in combination with EUS-FNA, over conventional bronchoscopic modalities in diagnosing Scadding stages I and II of the disease. Moreover, TBLC may be considered a useful and safe diagnostic tool for thoracic sarcoidosis, overcoming some limitations of transbronchial lung biopsy (TBLB), avoiding more invasive modalities and being complementary to endosonographic procedures such as EBUS-TBNA.
Diffuse lung diseases (DLD) are characterized by different immunophenotypes in the bronchoalveolar lavage fluid (BALF). We aimed to evaluate the diagnostic value of BALF NK and NKT cell counts of patients with DLD and lymphocytic alveolitis. We assessed 202 patients with DLD, who underwent BALF immunophenotyping. Samples were routinely processed by flow cytometry and lymphocyte subsets were compared between patients with sarcoidosis (n = 106), hypersensitivity pneumonitis (HP; n = 53), and other DLDs (n = 43). We compared absolute counts and percentages of NK and NKT cells between patients with HP versus the remaining DLD patients. To assess the accuracy of BALF lymphocyte subsets in the diagnosis of HP, we calculated the respective areas under the receiver operating characteristic curves (AUC-ROC). Patients with HP had significantly higher numbers of BALF NK cells, and its percentage was significantly associated with a higher odds of HP, even after adjustment for the NKT and CD8+ cells. For the absolute number of BALF NK cells, we found an AUC-ROC of 0.76 (95%CI = 0.68–0.84) when comparing patients with HP versus the remaining DLD. The cut-offs of 2000 NK cells/mL and of 2.4% NK cells in the BALF had a specificity and a negative predictive value over 80% for the diagnosis of HP. BALF NK cells absolute counts were significantly higher in HP patients with a restrictive pattern. No such differences were observed for NKT cells. BALF NK immunophenotyping may be a helpful adjunct to the diagnostic work-up of DLD, particularly in the differential diagnosis of HP.
30 July 2009 in Revista Portuguesa de Pneumologia (English Edition)
While open lung biopsy (OLB) performed in patients on mechanical ventilation (MV) with diffuse lung diseases (DLD) can be extremely important in establishing the diagnosis, the associated risk of this procedure should be taken into account. To determine the diagnostic yield, therapeutic changes, complications and mortality in patients with DLD on MV submitted to OLB. Retrospective study of 19 patients admitted to S. João Hospital between January 1999 and July 2007 (8.5 years). Data analysed included demographic data, ventilation variables before and after biopsy, diagnostic yield, effect on subsequent treatment changes and complications of OLB. Statistical analysis was performed using SPSS 14.0. The mean age of patients was 58+/-16.3 years old and 53% were male. The mean duration of hospital stay in Intensive Care Unit before performing OLB was 13+/-7 days. All biopsies were performed by thoracotomy. The diagnostic yield was 95%. There were no significant differences in partial pressure of arterial oxygen/fraction of inspired oxygen (PaO(2)/ FiO(2)) ratio and the positive end expiratory pressure (PEEP) before and after OLB. Postoperative complications occurred in 4 patients (21%; persistent air leak). Alteration in the diagnosis occurred in 14 patients (74%) and in 8 patients (42%) there was a modification in the treatment regimen. Global mortality was 47% (9 patients) but there were no biopsy-related deaths. The high diagnostic yield and the low incidence of complications make OLB a useful procedure in patients on MV with DLD of unknown aetiology. However, early OLB may lead to even better results in some patients.
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.
Introduction: In the past, pertussis affected particularly children under 6 years of age, but recent trends show that there is a shift toward the older age group. The clinical presentation can be atypical in the adolescent age group, and the disease is often misdiagnosed. Case presentation: We present a case of an 11-year-old male patient oriented to our unit with anorexia, weight loss and persistent cough with nocturnal paroxysms for 4 weeks. He also reported occasional wheezing and chest tightness. He denied fever, chills, myalgia, sore throat, or rhinorrhea. The patient presented to his primary care physician 1 week prior with the same complaint and was treated with amoxicillin and ebastine. Facing the persistence of the complaints he was oriented to our unit in order to exclude tuberculosis. Further study confirmed Bordetella pertussis infection and he started clarithromycin (15 mg/kg/day for 14 days). The patient's symptoms resolved after two weeks. Two of the patient's family members have developed symptoms of Bordetella pertussis infection and were treated after convenient study. Conclusion: Cough is one of the most common complaints among children and its causes are multiple. Active immunization and early diagnosis are crucial in the management of pertussis.