Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis characterised by inflammation of the small blood vessels, the absence of granulommas on histopathological specimens, with few or no immune deposits and the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCAs). The classic pulmonary manifestation is diffuse alveolar haemorrhage (DAH), but its association with pulmonary fibrosis (PF) has been increasingly reported and may be the first manifestation of MPA. Our aim was to evaluate MPA patients with PF and compare their characteristics and evolution to those of MPA patients without PF. We conducted a retrospective review of MPA patients followed in our hospital over a 15-year period. They were divided into two subgroups, with PF (MPA-PF) and without PF (MPA-non PF), and their clinical and functional features were compared. Nine of the 28 patients were classified as MPA-PF (32%). This subgroup showed significantly more respiratory symptoms and higher mortality than MPA-non PF subgroup. The most frequent chest computed tomographic pattern of PF was usual interstitial pneumonia. PF preceded other manifestations of vasculitis in five patients and occurred simultaneously in the remaining four. During the follow-up period, four deaths were reported in the MPA-PF subgroup. No deaths were registered in the MPA-non PF subgroup. We found a high prevalence of MPA-PF patients (32%), most of whom had a poor outcome and PF was often the first manifestation of the disease.
The diffuse cystic lung diseases (DCLDs) are a pathophysiologically heterogeneous processes characterized by the presence of multiple thin-walled, air-filled spaces within the pulmonary parenchyma. The most common causes of DCLD are lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH).DCLD develops rarely as a result of malignancy, typically secondary to metastases from peripheral sarcomas and mesenchymal tumors. DCLD have also been reported in a variety of other metastatic disease such as adenocarcinoma.Our case describes a patient with DCLD as a result of metastatic colorectal adenocarcinoma.
Differential diagnosis of lymphocytic pleural effusions between tuberculous (TBE) and malignant (ME) effusion is usually difficult in daily practice. Our aim was to develop a score to differentiate TBE from ME effusions. A cohort of 138 consecutive patients with pleural effusion was prospectively studied from May 2014 through June 2017. Glucose, lactate dehydrogenase (LDH), proteins, white cell count, lactic acid, and pH in the pleural fluid were measured. Pleural effusions other than lymphocytic, patients with a final diagnosis other than tuberculosis or malignancy, and patients who met Light’s criteria for transudate were excluded. Eighty-two samples (47 TBE and 35 ME) were included in the analysis. Using logistic regression analysis and Wald test, we developed a score including age, glucose, proteins, and lactic acid. The receiver operating characteristic curve (ROC) for the score was determined, and the area under the curve (AUC) was calculated. A cutoff of eight points was required to achieve 93.5% sensitivity, 78% specificity, and a likelihood ratio of 4.26 to distinguish tuberculosis from malignant pleural effusion. The AUC of the score was 0.915 (95% CI = 0.82–0.96).
The utility of the adenosine deaminase (ADA) assay in the diagnosis of patients with pleural tuberculosis (TB) and human immunodeficiency virus (HIV) infection is controversial. Forty-eight HIV positive patients with pleural effusion were evaluated; ADA assay was obtained in forty-three of them. Twenty-five patients presented diagnosis of TB. Patients with diagnosis of TB showed a median value of ADA of 70 IU/L (interquartile range (IQR) 41–89) and the non-TB group a median of 27.5 IU/L (IQR 13.5–52). Patients with diagnosis of TB had a median cluster of differentiation 4 (CD4) count of 174 (IQR 86–274) and the non-TB group had a median of 134 (IQR 71–371). Receiver operating characteristic curve was performed with an area under the curve of 0.79. The best cut-off obtained was 35 IU/L with a sensibility of 80% and a specificity of 66%. There was no correlation between CD4 lymphocytes count and the value of ADA in the TB patient group.
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