Respiratory function in patients with pemphigus vulgaris - a small clinical study.

BACKGROUND: Pemphigus vulgaris belongs to a group of rare, autoimmune, blistering disorders, requiring intensive immunosuppressive therapy. Lung infections are common in patients with pemphigus vulgaris. OBJECTIVE: To evaluate pulmonary function in patients with pemphigus vulgaris, in order to detect possible factors that might predispose PV patients to lung damage. METHODS: Ten patients first diagnosed with severe pemphigus vulgaris and 10 healthy individuals,were included in the study. Pulmonary function testing, blood gas analysis and quantification of α1-antitrypsin serum levels were performed. RESULTS: Mild reduction of CO diffusing capacity was recorded, compared to the mean predicted normal value. DLCOSB (single-breath carbon monoxide diffusing capacity test) values did not significantly differ between patients with pemphigus vulgaris and healthy controls, while differences regarding DLCO/VA (VA: Alveolar Volume) were statistically significant. Alpha 1-antitrypsin serum levels were decreased (<2.0g/L) in 60% (6/10) of patients with pemphigus vulgaris and were found normal in none of the healthy controls. CONCLUSION: A mild reduction of pulmonary diffusing capacity was observed in patients with pemphigus vulgaris.

Assessment of respiratory mechanics by impulse oscillometry in orthopneic patients with acute left ventricular failure.

BACKGROUND: This study investigated the respiratory function and mechanics of patients with orthopnea caused by acute left ventricular failure (ALVF). METHODS: The study comprised 40 patients with ALVF and 15 control subjects. All patients underwent lung function tests and impulse oscillometry in both sitting and supine positions. In a subgroup of 22 patients, isosorbide dinitrate was administered and impulse oscillometry was performed 15 minutes later in the supine position. RESULTS: No patient reported dyspnea while seated, and the orthopnea score was 2.9 ± 1.4. Left ventricular ejection fraction was 43% ± 10%. Patients demonstrated restrictive spirometric pattern in the sitting position, whereas functional residual capacity was comparable to that of the control group. In the supine position, all pulmonary volumes decreased, except inspiratory capacity which increased. Respiratory reactance (Xrs5) was higher in patients in both sitting (421.8 ± 630.6%pred vs 147.2 ± 72.8%pred, P = .01) and supine (699.8 ± 699.9%pred vs 251.2 ± 151.6%pred, P ≤ .001) positions. Respiratory resistance (Rrs5) (10.6% ± 17.8% mean decrease) and Xrs5 (17.2% ± 39.4% mean decrease) improved after nitrates administration. Orthopnea was better correlated with Xrs5%pred in the supine position (r = .42, P = .007). Ejection fraction was positively correlated with inspiratory capacity %pred (r = .42, P = .007) in the sitting position. CONCLUSION: Patients with ALVF demonstrated increased respiratory reactance that correlated with orthopnea severity and improved after nitrates administration.

Efficacy and safety of erythromycin as sclerosing agent in patients with recurrent malignant pleural effusion.

OBJECTIVES: The aim of pleurodesis in malignant pleural effusions is to prevent reaccumulation of the fluid, symptoms, and avoid the need for repeated hospitalization for thoracentesis. The purpose of this study was to evaluate the efficacy and safety of erythromycin as a pleural sclerosing agent. METHODS: Over a 2-year period, 34 patients with a symptomatic, recurrent, malignant pleural effusion who referred for chest tube drainage and pleurodesis were included. They had not received prior intrapleural therapy and had predicted survival of at least 1 month. All underwent pleural drainage and chemical pleurodesis with erythromycin. Complications and response to pleurodesis, according to clinical and radiographic criteria after 90 days, were recorded. RESULTS: The overall response was 88.2%. Complete response (no reaccumulation of pleural fluid after 90 days) was observed in 27 patients (79.4%). Partial response (reaccumulation of fluid but without symptoms, not requiring drainage) was observed in 3 (8.8%). No response (symptomatic reaccumulation of fluid that required drainage) was observed in 4 (11.8%). All patients experienced pleurodynia that was treated with administration of paracetamol and/or dextropropoxyphene. Sinus tachycardia and concurrent mild systemic hypertension were observed 2 and 4 hours after pleurodesis. Both of them were attributed to pleurodynia as there was remission with analgesics. CONCLUSIONS: This study suggests that erythromycin is effective and safe as a sclerosing agent for pleurodesis in patients with recurrent malignant pleural effusions.

Epstein-Barr virus DNA in bronchoalveolar lavage fluid from patients with idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis is the most common idiopathic interstitial pneumonia. The human herpesviruses and especially Epstein-Barr virus have been implicated in the etiology of idiopathic pulmonary fibrosis in a number of studies. AIM: The aim of this study was to investigate the potential association between idiopathic pulmonary fibrosis and Epstein-Barr virus. METHODS: Bronchoalveolar lavage fluid and sera were collected from 63 patients out of whom 17 suffered of idiopathic pulmonary fibrosis and 46 of other interstitial lung diseases. Sera from 50 healthy, age-matched individuals were also collected. Antibodies to the early, nuclear, and capsid antigens of Epstein-Barr virus were determined by enzyme immunoassay and indirect immunofluorescence. Additionally polymerase chain reaction was performed in bronchoalveolar lavage fluid in order to investigate the presence of Epstein-Barr virus DNA. Positive polymerase chain reaction results were confirmed by nucleotide sequencing. RESULTS: Statistically significant differences were observed in the frequency of IgA antibodies to viral capsid antigen among patients with idiopathic pulmonary fibrosis, patients with other interstitial lung diseases and healthy controls (60%, 24.4% and 22% respectively, p = 0.013). Epstein-Barr virus DNA was detected in the bronchoalveolar lavage fluid of 3 patients with idiopathic pulmonary fibrosis but in none of the patients with other diseases (p = 0.024). CONCLUSIONS: The results of this study support the association between IPF and EBV, at least in some cases, and provide evidence that BALF is an alternative for the detection of viral DNA in patients with IPF. However further investigation is required concerning the etiology of idiopathic pulmonary fibrosis.

Variations in alveolar cell populations, lymphocyte subsets and NK-cells in different stages of active pulmonary sarcoidosis.

BACKGROUND: The type and mode of presentation of pulmonary sarcoidosis (radiologic stage, activity) influence alveolar lymphocyte number and subsets as well as other inflammatory cells. AIM: To investigate the variations in alveolar cells, lymphocyte subsets and NK-cells in different radiological stages of active pulmonary sarcoidosis. METHODS: 85 untreated, newly diagnosed patients (32 male, 53 female), median age 47.2+/-14.7 years were investigated. Patients were classified into chest x-ray stages (33 stage I, 27 stage II, 14 stage III and 11 stage IV disease). Bronchoalveolar lavage was performed with 4 portions of 50 mL. Total cells and cell differentials were counted, while CD3+, CD4+, CD8+, CD19+, CD3-CD16/56+ (NK-cells) and CD3+CD16/56+ (NKT-cells) were determined by flow cytometry. RESULTS: Lymphocytosis was high in all stages. Significant differences were found in: a) CD4 between stages I and II and I and IV. b) CD8 between stages I and IV and c) NK and NKT cells between stages I and III. CONCLUSIONS: Variations were observed in alveolar cells and lymphocyte subsets in different stages of pulmonary sarcoidosis. Cells exhibiting cytotoxic activity were increased with stage progression, CD8 from stage I to IV, NK and NKT cells from stage I to III. These cells may be involved in the inflammatory process regulating granuloma formation.

Factors influencing patient survival and renal function outcome in pulmonary-renal syndrome associated with ANCA (+) vasculitis: a single-center experience.

Small vessel vasculitides, usually associated with positive antineutrophil cytoplasmic antibodies (ANCA(+)) can cause pulmonary-renal syndrome (PRS). Data from 22 patients, (19 males), aged 28-76 yrs (mean 55), with PRS were analyzed retrospectively. Renal function was estimated at presentation, 1 month after treatment initiation and at the end of follow-up (mean 4.4 +/- 3.3 yrs). Thirteen out of 22 patients had PR3 (+) ANCA and 9/22 patients had MPO (+) ANCA. Mean serum creatinine (Cr) at diagnosis was 6.6 +/- 4.4 mg/dL (M +/- SD) and proteinuria 1.6 +/- 1.4 g/24 hr (M +/- SD). During the 1st month of treatment with corticosteroids and cyclophosphamide, renal function improved in 12 patients (54.5%) (serum Cr from 8.5 +/- 4.5 to 4.3 +/- 2.3 mg/dL, p=0.001) remaining stable thereafter, and renal function deteriorated in nine patients (41%) (serum Cr from 4.1 +/- 3 to 6.5 +/- 2.9 mg/dL, p=0.03); one patient (4.5%) died because of sepsis. At the end of the study, 11/22 patients (50%) had died, eight patients of these (73%) because of respiratory failure, three patients (13.6%) reached end-stage renal disease (ESRD), five patients (36.4%) remained stable, but with impaired renal function and finally three patients (13.6%) improved, achieving almost normal renal function. In multiple regression analysis, factors contributing to final serum Cr were: dialysis dependency at the time of diagnosis p=0.01, initial proteinuria p<0.0001, and percentage of cellular crescents p=0.003. Main differences between PR3 and MPO (+) patients were the chest CT findings (bilateral nodules in PR3 (+) and "ground glass" or fibrosis in MPO (+) patients) and the renal function improvement rate after treatment initiation (rapid decline in serum Cr in PR3 (+) patients). In conclusion, PRS with ANCA (+) is associated with increased mortality. If renal function improves during the 1st month of treatment, it usually remains stable thereafter. The presence of PR3-ANCA is associated with an early response to treatment, while MPO-ANCA vasculits seems to necessitate prolonged treatment.

Fifteen years' record of airborne allergenic pollen and meteorological parameters in Thessaloniki, Greece.

A pollen calendar has been constructed for the area of Thessaloniki and relationships between pollen transport and meteorological parameters have been assessed. Daily airborne pollen records were collected over a 15-year period (1987-2001), using a Burkard continuous volumetric pollen trap, located in the centre of the city. Sixteen allergenic pollen types were identified. Simultaneously, daily records of five main meteorological parameters (mean air temperature, relative humidity, rainfall, sunshine, wind speed) were made, and then correlated with fluctuations of the airborne pollen concentrations. For the first time in Greece, a pollen calendar has been constructed for 16 pollen types, from which it appears that 24.9% of the total pollen recorded belong to Cupressaceae, 20.8% to Quercus spp., 13.6% to Urticaceae, 9.1% to Oleaceae, 8.9% to Pinaceae, 6.3% to Poaceae, 5.4% to Platanaceae, 3.0% to Corylus spp., 2.5% to Chenopodiaceae and 1.4% to Populus spp. The percentages of Betula spp., Asteraceae (Artemisia spp. and Ambrosia spp.), Salix spp., Ulmaceae and Alnus spp. were each lower than 1%. A positive correlation between pollen transport and both mean temperature and sunshine was observed, whereas usually no correlation was found between pollen and relative humidity or rainfall. Finally, wind speed was generally found to have a significant positive correlation with the concentrations of 8 pollen types. For the first time in the area of Thessaloniki, and more generally in Greece, 15-year allergenic pollen records have been collected and meteorological parameters have been recorded. The airborne pollen concentration is strongly influenced by mean air temperature and sunshine duration. The highest concentrations of pollen grains are observed during spring (May).