Hypersensitivity pneumonitis (HP) is the third, according to frequency, interstitial lung disease, with the estimated incidence rate of 1-2/100,000. In HP patients, the extensive inflammatory lesions ...encompassing both small airways and lung parenchyma, as well as subsequent development of lung fibrosis, may result in respiratory insufficiency and secondary pulmonary hypertension (PH). The aim of the present retrospective study was to assess the prevalence of echocardiographic signs of PH and its' clinical predictors, in newly recognized HP patients.
Consecutive HP patients, recognized in single pulmonary unit between 2005 and 2017, in whom echocardiography was performed at diagnosis, entered the present study. HP diagnosis was verified in every patient according to current diagnostic recommendations. The results of high resolution computed tomography of the chest (HRCT) were re-evaluated by two independent radiologists, blinded to clinical data. Echocardiographic signs of PH were defined as pulmonary artery systolic pressure (PASP) exceeding 36 mmHg. Regression analysis was applied to calculate PH risk, and receiver operator characteristic curves (ROC) were plotted to investigate diagnostic utility of various parameters in PH prediction.
PASP exceeding 36 mmHg was noted in 26 out of 70 patients (37%)-with equal frequency among patients with fibrotic and non-fibrotic HP. Significant predictors of PH on echocardiography were: partial oxygen tension in arterialized capillary blood (PaO2) <69 mmHg, lung transfer capacity for carbon monoxide (TLCO) <42% of predicted, six minutes walking test (6MWT) distance <455 meters, and 6MWT desaturation rate >8%. In case of TLCO <42% of predicted, probability of PH on echocardiography was increased by five-fold, in case of 6MWT desaturation rate >8%-by four fold.
The best predictors of PASP >36 mmHg on echocardiography in HP patients at diagnosis were: TLCO <42% and 6MWT desaturation rate >8%. Neither the presence of lung fibrosis on HRCT, nor the duration of the disease or patients age, were helpful in PH prediction.
Idiopathic pulmonary fibrosis (IPF) and chronic hypersensitivity pneumonitis share commonalities in pathogenesis shifting haemostasis balance towards the procoagulant and antifibrinolytic activity. ...Several studies have suggested an increased risk of venous thromboembolism in IPF. The association between venous thromboembolism and chronic hypersensitivity pneumonitis has not been studied yet.
A retrospective cohort study of IPF and chronic hypersensitivity pneumonitis patients diagnosed in single tertiary referral center between 2005 and 2018 was conducted. The incidence of symptomatic venous thromboembolism was evaluated. Risk factors for venous thromboembolism and survival among those with and without venous thromboembolism were assessed.
A total of 411 (259 IPF and 152 chronic hypersensitivity) patients were included (mean age 66.7 ± 8.4 vs 51.0 ± 13.3 years, respectively). There were 12 (4.6%) incident cases of venous thromboembolism in IPF and 5 (3.3%) in chronic hypersensitivity pneumonitis cohort. The relative risk (RR) of venous thromboembolism in chronic hypersensitivity pneumonitis was not significantly different to that found in patients with IPF (7.1 vs 11.8/1000 person-years, RR 1.661 95% CI 0.545-6.019, respectively). The treatment with systemic steroids (OR 5.38; 95% CI 1.65-18.8, p = 0.006) and GAP stage 3 (OR 7.85; 95% CI 1.49-34.9; p = 0.037) were significant risk factors for venous thromboembolism in IPF. Arterial hypertension and pulmonary hypertension significantly increased risk of venous thromboembolism in chronic hypersensitivity pneumonitis. There were no significant differences in survival between patients with and without venous thromboembolism.
The patients with chronic hypersensitivity pneumonitis have a marked increase in the risk of venous thromboembolism, similar to the patients with IPF. Venous thromboembolism does not affect the survival of patients with IPF and chronic hypersensitivity pneumonitis.
The six-minute-walking test (6MWT) is an easy-to-perform, cheap and valuable tool to assess the physical performance of patients. It has been used as one of the endpoints in many clinical trials ...investigating treatment efficacy in pulmonary arterial hypertension and idiopathic pulmonary fibrosis. However, the utility of 6MWT in patients diagnosed with hypersensitivity pneumonitis (HP) is still under investigation. The aim of the present retrospective study was to assess the value of different 6MWT parameters, including the newly developed distance-desaturation index (DDI), to evaluate immunomodulatory treatment outcomes in HP patients.
6MWT parameters (distance, initial saturation, final saturation, desaturation, distance-saturation product (DSP), and DDI) were analyzed at baseline and after 3 to 6 months of treatment with corticosteroids alone or in combination with azathioprine.
91 consecutive HP patients diagnosed and treated in a single pulmonary unit from 2005 to 2017 entered the study. There were 44 (48%) males and 52 (57%) patients with fibrotic HP (fHP). Sixty-three patients (69%) responded to treatment (responders) and 28 (31%) did not respond (non-responders). In the responders group, all parameters assessed during 6MWT significantly improved, whereas in non-responders, they worsened. Medians (95% CI) of best indices were post-treatment DDI/baseline DDI-1.67 (1.85-3.63) in responders versus 0.88 (0.7-1.73) in non-responders (
= 0.0001) and change in walking distance-51 m (36-72 m) in responders, versus 10.5 m (-61.2-27.9) in non-responders (
= 0.0056). The area under the curve (AUC) of receiver operating characteristics (ROC) for post-treatment DDI/baseline DDI was 0.74 and the optimal cut-off was 1.075, with 71% of specificity and 71% of sensitivity.
6MWT may be used as a tool to assess and monitor the response to immunomodulatory therapy in HP patients, especially if indices incorporating both distance and desaturation are used. Based on the present study results, we recommend 6MWD and DDI use, in addition to FVC and TL,co, to monitor treatment efficacy in patients with interstitial lung diseases.
Hypersensitivity pneumonitis (HP) is one of the interstitial lung diseases with clearly established diagnostic criteria. Nevertheless, pharmacologic treatment recommendations are still lacking. Most ...specialists use steroids as first-line drugs, sometimes combined with an immunosuppressive agent. Aim: The aim of the present retrospective study was to establish predictive factors for treatment success and survival advantage in HP patients. Methods: We analyzed the short-term treatment outcome and overall survival in consecutive HP patients treated with prednisone alone or combined with azathioprine. Results: The study group consisted of 93 HP patients, 54 (58%) with fibrotic HP and 39 (42%) with non-fibrotic HP. Mean (± SD) VCmax % pred. and TL,co % pred. before treatment initiation were 81.5 (±20.8)% and 48.3 (±15.7)%, respectively. Mean relative VCmax and TL,co change after 3−6 months of therapy were 9.5 (±18.8)% and 21.4 (±35.2)%, respectively. The short-term treatment outcomes were improvement in 49 (53%) patients, stabilization in 16 (17%) patients, and progression in 28 (30%) patients. Among those with fibrotic HP, improvement was noted in 19 (35%) cases. Significant positive treatment outcome predictors were fever after antigen exposure, lymphocyte count in broncho-alveolar lavage fluid (BALF) exceeding 54%, RV/TLC > 120% pred., and ill-defined centrilobular nodules in high-resolution computed tomography (HRCT). An increased eosinophil count in BALF and fibrosis in HRCT were significant negative treatment outcome predictors. The presence of fibrosis in HRCT remained significant in a multivariate analysis. A positive response to treatment, as well as preserved baseline VCmax (% pred.) and TLC (% pred.), predicted longer survival, while fibrosis in HRCT was related to a worse prognosis. Conclusion: Immunomodulatory treatment may be effective in a significant proportion of patients with HP, including those with fibrotic changes in HRCT. Therefore, future trials are urgently needed to establish the role of immunosuppressive treatment in fibrotic HP.
Fibrotic hypersensitivity pneumonitis (fHP) shares many features with other fibrotic interstitial lung diseases (ILD), and as a result it can be misdiagnosed as idiopathic pulmonary fibrosis (IPF). ...We aimed to determine the value of bronchoalveolar lavage (BAL) total cell count (TCC) and lymphocytosis in distinguishing fHP and IPF and to evaluate the best cut-off points discriminating these two fibrotic ILD.
A retrospective cohort study of fHP and IPF patients diagnosed between 2005 and 2018 was conducted. Logistic regression was used to evaluate the diagnostic utility of clinical parameters in differentiating between fHP and IPF. Based on the ROC analysis, BAL parameters were evaluated for their diagnostic performance, and optimal diagnostic cut-offs were established.
A total of 136 patients (65 fHP and 71 IPF) were included (mean age 54.97 ± 10.87 vs. 64.00 ± 7.18 years, respectively). BAL TCC and the percentage of lymphocytes were significantly higher in fHP compared to IPF (
< 0.001). BAL lymphocytosis >30% was found in 60% of fHP patients and none of the patients with IPF. The logistic regression revealed that younger age, never smoker status, identified exposure, lower FEV
, higher BAL TCC and higher BAL lymphocytosis increased the probability of fibrotic HP diagnosis. The lymphocytosis >20% increased by 25 times the odds of fibrotic HP diagnosis. The optimal cut-off values to differentiate fibrotic HP from IPF were 15 × 10
for TCC and 21% for BAL lymphocytosis with AUC 0.69 and 0.84, respectively.
Increased cellularity and lymphocytosis in BAL persist despite lung fibrosis in HP patients and may be used as important discriminators between IPF and fHP.
Hypersensitivity pneumonitis (HP) is an increasingly recognized interstitial lung disease, presenting with elevated total cell counts and high percentage of lymphocytes in bronchoalveolar lavage ...fluid (BALF). Despite many publications, there is no consensus in the literature concerning BALF cellular composition in patients with prolonged course of HP.
The aim of the present retrospective study was to investigate the influence of disease duration, smoking habits, and the extent of lung fibrosis on BALF cells' population in patients with newly recognized HP.
In total, 94 patients (49 females, 45 males), mean age 52 (±12) years, with HP recognized according to recently proposed criteria, were enrolled into the present study. Chest CT scans were retrospectively reviewed by two independent radiologists. BALF evaluation was performed as a part of routine diagnostics according to recent recommendations.
Percentage of lymphocytes in BALF was significantly lower in patients with lung fibrosis (stage 1 and 2) comparing to those without lung fibrosis (stage 0). Significant correlation was also found between the percentage of BALF lymphocytes and plethysmographic lung volumes, but not with lung transfer capacity for carbon monoxide (TLCO% pred). Smoking did not influence BALF results in our study group.
BALF lymphocytosis correlated with the presence and the extent of lung fibrosis on chest CT as well as with plethysmographic lung volumes but did not with TLCO and smoking habits in newly recognized HP pneumonitis.
Our aim was to compere diabetes-related distress (DD) in young patients with type 1 diabetes mellitus (T1DM) and in their parents before and during the national COVID-19-related lockdown when schools ...operated on-line. Problems Areas in Diabetes-Child (PAID-Ch), Teen (PAID-T) and Parent (P-PAID-Ch, P-PAID-T) questionnaires in paper version were used to evaluate DD before COVID-19 pandemic (November 2019–February 2020) and during the lockdown (April 2020) the same surveys were performed by phone. We enrolled 76 patients (median age (Q1–Q3): 13.6 (11.8–15.2) years; 21 children, 55 adolescents; T1DM duration 3.7 (1.7–6.8) years). Initial PAID score was lower in teenage boys than in girls (34.0 (24.0–42.0) vs. 44.5 (40.0–50.5), p = 0.003). In teens PAID score decreased significantly during the lockdown (−3.0 (−11.0–3.0), p = 0.018), more in girls than boys (p = 0.028). In children (−3.0 (−14.0–7.0), p = 0.131) and parents PAID did not change (teens’ parents: 3.0 (−9.0–10.0), p = 0.376; children’s parents: −5.0 −9.0–1.0, p = 0.227). In the studied group COVID-19 pandemic-related lockdown was associated with decrease in DD in teens with T1DM, particularly in girls, while no significant change in DD was observed in children or parents. DD decrease in teens during the pandemic should attract attention to the potential “rebound” of DD related to return to regular on-site school routine.
Hypersensitivity pneumonitis (HP) is the third most common interstitial lung disease after idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Pathogenesis of HP is related to ...repeated exposure to inhaled environmental antigens that sensitise the susceptible, genetically predisposed persons. The aim of the present retrospective study was to summarise the diagnostic methods used in consecutive patients with HP, recognised in a single pulmonary unit, between 2005 and 2015, and to compare them with current diagnostic criteria.
135 patients, 68 males, 67 females, median age 53 years (18-75 years), entered the study. Chest CT features characteristic of HP were defined as: mosaic attenuation of lung parenchyma, air trapping and/or ill-defined centrilobular nodules. Lymphocytosis in BAL was defined as ≥ 30%.
Median time from first symptoms to diagnosis was 12 months. The exposure to one or more allergens was found in 94% of patients, chest CT features characteristic of HP have been reported in 87%, BAL lymphocytosis - in 86%. According to recent diagnostic criteria - in 54% of patients, clinical diagnosis of HP was confident, in 16% - probable, in 26% - possible and in 4% - unlikely. The confirmation of HP with lung biopsy has been obtained in 36% of non-confident cases (16% of the study group).
HP diagnosis was confirmed according to current diagnostic criteria in 70% of patients diagnosed between 2005 and 2015. Contradictions to lung biopsy have been the main reason for inability to confirm HP in non-confident cases.
Intra-vesical instillations with bacillus Calmette-Guerin (BCG) are the established adjuvant therapy for superficial bladder cancer. Although generally safe and well tolerated, they may cause a range ...of different, local, and systemic complications. We present a patient treated with BCG instillations for three years, who was admitted to our hospital due to fever, hemoptysis, pleuritic chest pain and progressive dyspnea. Chest computed tomography (CT) showed massive bilateral ground glass opacities, partly consolidated, localized in the middle and lower parts of the lungs, bronchial walls thickening, and bilateral hilar lymphadenopathy. PCR tests for SARS-CoV-2 as well as sputum, blood, and urine for general bacteriology-were negative. Initial empiric antibiotic therapy was ineffective and respiratory failure progressed. After a few weeks, a culture of
complex was obtained from the patient's specimens; the cultured strain was identified as
BCG. Anti-tuberculous treatment with rifampin (RMP), isoniazid (INH) and ethambutol (EMB) was implemented together with systemic corticosteroids, resulting in the quick improvement of the patient's clinical condition. Due to hepatotoxicity and finally reported resistance of the BCG strain to INH, levofloxacin was used instead of INH with good tolerance. Follow-up CT scans showed partial resolution of the pulmonary infiltrates. BCG infection in the lungs must be taken into consideration in every patient treated with intra-vesical BCG instillations and symptoms of protracted infection.
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