New 6-Minute-Walking Test Parameter-Distance/Desaturation Index (DDI) Correctly Diagnoses Short-Term Response to Immunomodulatory Therapy in 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. METHODS: 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. RESULTS: 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 (p = 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 (p = 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. CONCLUSIONS: 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.

Bronchoalveolar Lavage Cell Count and Lymphocytosis Are the Important Discriminators between Fibrotic Hypersensitivity Pneumonitis and Idiopathic Pulmonary Fibrosis.

BACKGROUND: 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. METHODS: 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. RESULTS: 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 (p < 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 FEV1, 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 × 106 for TCC and 21% for BAL lymphocytosis with AUC 0.69 and 0.84, respectively. CONCLUSIONS: Increased cellularity and lymphocytosis in BAL persist despite lung fibrosis in HP patients and may be used as important discriminators between IPF and fHP.

Factors Predictive for Immunomodulatory Therapy Response and Survival in Patients with Hypersensitivity Pneumonitis-Retrospective Cohort Analysis.

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.

Severe Respiratory Failure Due to Pulmonary BCGosis in a Patient Treated for Superficial Bladder Cancer.

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 M. tuberculosis complex was obtained from the patient's specimens; the cultured strain was identified as Mycobacterium bovis 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.

Chronic hypersensitivity pneumonitis is associated with an increased risk of venous thromboembolism: a retrospective cohort study.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Echocardiographic signs of pulmonary hypertension in patients with newly recognized hypersensitivity pneumonitis, prevalence and clinical predictors.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Polymetastatic testicular cancer turns out to be secondary syphilis: a case report.

Syphilis is a sexually transmissible infection, with increasing rates of infection worldwide. The differential diagnosis of syphilis should include various diseases, not excluding cancer. Making the right diagnosis can protect the patient against life-threatening complications and the repercussions of a misdiagnosis, as in the present case (orchidectomy).

Correlation of bronchoalveolar lavage lymphocyte count with the extent of lung fibrosis and with plethysmographic lung volumes in patients with newly recognized hypersensitivity pneumonitis.

INTRODUCTION: 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. AIM: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Hypersensitivity pneumonitis recognised in a single pulmonary unit, between 2005 and 2015 - comparison with recently proposed diagnostic criteria.

INTRODUCTION: 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. MATERIAL AND METHODS: 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%. RESULTS: 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). CONCLUSION: 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.

Morphology and morphometry of pulmonary veins and the left atrium in multi-slice computed tomography.

PURPOSE: Pulmonary veins are involved in pathogenesis and treatment of atrial fibrillation and structures at risk during thoracic surgeries. There is lack of data regarding pulmonary vein morphology and morphometry in normal population. METHODS: The study was conducted using 135 chest computed tomography studies with intra-venous iodine contrast injection. The study population contained 86 females and 49 males, mean age was 60. 13 had atrial fibrillation.The studies were analyzed using radiological workstation. RESULTS: Mean dimensions of the left atrium: transverse 52 mm, coronal 49 mm, and sagittal 35 mm. The mean volume of the left atrium was 93 cm3. The mean volume of the left atrium in patients with atrial fibrillation was 176 cm3. The sagittal dimension and the volume of the left atrium were correlated with age, r = 0.43 and r = 0.42, respectively. Surface area of the left inferior pulmonary vein ostium was 136 mm2, significantly less than the surface area of other ostia of pulmonary veins. The mean distance between two pulmonary veins was 5.42 mm on the right and 4.02 mm on the left side. 13 types of pulmonary veins outflow patterns were described on the right side and 5 types on the left side. 66.7% of right pulmonary veins and 82% of the left pulmonary veins emptied into the left atrium with two venous trunks on each side (the typical pattern). CONCLUSIONS: Morphological features of pulmonary veins and morphometry of the left atrium and pulmonary veins are important for clinical purposes and are in accordance with previous papers.

Clinical presentation and characteristics of 25 adult cases of pulmonary sequestration.

BACKGROUND: Pulmonary sequestration (PS) is a rare congenital abnormality of lung tissue. Only few series of adult cases are reported. The aim was to describe clinical characteristics in adult cases of PS and to compare outcomes in different clinical situations. METHODS: Using MSD engine we searched for cases of PS that have been diagnosed between Jan 1st, 2005 and Dec 31st, 2015. Clinical data was retrospectively gathered. Statistica v.12 (StatSoft, Inc.) was used for statistical analyses. RESULTS: We found 25 cases (18 females, 7 males), which underwent surgery and were histologically proven. There were 22 cases of intralobar PS. 7 cases were asymptomatic, 12 had infectious history (including 3 cases of lung abscess and pleural empyema), 4 presented with hemoptysis, 2 with chest pain. The average age to undergo surgery was 38.24, in the asymptomatic group 34, in symptomatic 39.89. In the latter the symptoms preceded the surgery for 2.45-year. Great majority of sequestrations was located in lower lobes (96%), 52% on the left. Symptomatic cases were at higher than expected risk of surgical complications, comparing to asymptomatic (chi2, P=0.04). In most cases there were surgical and histological signs of infection, only in 9 cases etiological factor was determined: in 5 cases it was A. fumigatus. A 0.53-day longer post-surgical hospital stay was observed in the symptomatic group, no statistical significance was found (U-test, P=0.45). CONCLUSIONS: Surgical treatment of symptomatic cases of PS is characterized by slightly longer post-surgical hospital stay and higher risk of surgical complications. Fungal infections are the most likely to occur in PS.

Non-high risk PE in the patients with acute or exacerbated respiratory disease: the value of the algorithm based on D-dimer evaluation and Revised Geneva Score.

INTRODUCTION: The diagnostic algorithm of non-high risk pulmonary embolism (PE) is based on probability scoring systems and plasma D-dimer (DD) assessment. The aim of the present study was to investigate the efficacy of Revised Geneva Scoring (RGS) and DD testing for the excluding of non-high risk PE, in the patients admitted to the hospital due to acute respiratory diseases. MATERIAL AND METHODS: The consecutive patients, above 18 years of age, referred to the department of lung diseases, entered the study. The exclusion criteria were: the pregnancy and the suspicion of high risk PE. Plasma DD was measured with quick ELISA test, VIDAS D-dimer New, bioMerieux, France. Multislice computed tomography angiography was performed in all of the patients. RESULTS: 153 patients, median age 65 (19-88) years entered the study. The probability of PE was: low - in 58 patients (38%), intermediate - in 90 (59%), high - in 5 (3%). DD < 500 ng/ml was found in 12% of patients with low and intermediate probability of PE. PE was recognized in 10 out of 153 patients (7%). None of the patients with DD < 500 ng/ml was diagnosed with PE (NPV 100%). Median DD value was significantly higher in PE patients comparing to non-PE (4500 ng/ml and 1356 ng/ml respectively, p = 0.006). CONCLUSION: In the group of the patients with acute respiratory symptoms, low or intermediate clinical probability scoring combined with normal DD had a high NPV in excluding PE. Nevertheless, such approach was not very effective, as the increased DD was noted in 88% of the examined population.

Methotrexate as a single agent for treating pulmonary sarcoidosis: a single centre real-life prospective study.

INTRODUCTION: The first-line therapy in chronic sarcoidosis, according to WASOG/ATS/ERS recommendations, is GCS. This therapy is associated with significant adverse effects and finally does not alter the natural history of the disease. The objective of our study was to evaluate the efficacy and safety of monotherapy with MTX, as an alternative to GCS, in progressive pulmonary sarcoidosis. MATERIAL AND METHODS: An open prospective real-life, single-centre trial was performed on 50 patients with biopsy proven sarcoidosis, 28M and 22F, mean age 45.55 ± 8.9 years. The average duration of disease before MTX therapy was 12.34 ± 20.49 years, GCS therapy in the past was applied in 41 patients. All patients received MTX (10 mg or 15 mg weekly) between 2004 and 2013 because of chronic progressive pulmonary sarcoidosis. Therapy was planned for 24 months. Patients underwent regular clinical evaluation, pulmonary function assessment, exercise ability testing (6MWT), and chest radiography for therapy effectiveness every six months and side effects monitoring every 4-6 weeks. Forty-nine patients were included for statistical analysis of treatment efficacy. They were retrospectively allocated to "MTX responder" group if an improvement of 10% of FEV1, FVC, TLC, or 15% of DLCO from the initial value was documented for at least one parameter or "non-responders" if the patient did not meet the above-mentioned criteria. RESULTS: Duration of treatment ranged from 6 to 24 months, mean time 60.75 ± 34.1 weeks. For the whole cohort significant improvement after MTX therapy was observed for minimal SaO2 (%) (p = 0.043) and for decrease of DSaO2 (%) (p = 0.048) in six-minute walk test. The results were significantly better for patients treated with 15 mg than for those treated with 10 mg weekly and for those who obtained a greater total amount of MTX during therapy. Significant difference of DLCO%pred was observed after six months of MTX therapy between groups treated 15 mg vs 10 mg weekly (73.27 ± 12.7% vs. 63.15 ± 16.4%, p = 0.03). Twenty-five patients (55%) met the criteria of "MTX responders" group. Patients who responded well to treatment had significantly lower TLC and FVC initial values comparing to "MTX non-responders". After treatment the only significant difference in PFT between groups was noted for DLCO%pred. Eleven patients (22%) stopped the treatment due to adverse events of MTX, mild hepatic abnormalities were observed in ten patients (20%), and concomitant infection was found in four patients. There were no patients with a fatal outcome. CONCLUSIONS: MTX as a single agent in the treatment of sarcoidosis has proved to be a safe and effective steroid alternative. Selected patients with chronic pulmonary sarcoidosis experience definite PFT improvements after MTX treatment. There is need to search for predictors of MTX treatment effectiveness.

[Interstitial pneumonia connected with rituximab therapy--case report]. / Sródmiazszowe zapalenie pluc zwiazane z leczeniem rytuksymabem--opis przypadku.

Rituximab (RTX) is a monoclonal antibody against the CD20 antigen found on the surface of B cells. RTX causes cell lysis and is therefore used to treat lymphomas, leukaemias, transplant rejection and certain autoimmune disorders. Pulmonary adverse events associated with RTX have been reported in literature. We describe a patient with follicular lymphoma treated with R-CHOP scheme (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) who had pulmonary symptoms during chemotherapy which were diagnosed as rituximab-induced interstitial pneumonia. Symptoms and radiological changes resolved with prednisone therapy.

Assessment of recurrence of non-small cell lung cancer after therapy using CT and Integrated PET/CT.

INTRODUCTION: Non-small cell lung cancer (NSCLC) has become the leading cause of cancer-related deaths in Poland. Follow-up of patients with NSCLC is aimed at early detection of local recurrence, metastatic process, treatment-related complications or second primary lung cancer. We investigated the diagnostic accuracy of FDG-PET-CT in the detection of recurrence of NSCLC after treatment. MATERIAL AND METHODS: Seventy-two NSCLC patients (19 females, 56 males), stage I to IV, who had undergone surgery and/ /or radiation therapy, occasionally associated with chemotherapy, were retrospectively included in our study. Chest radiographs and thoracic computed tomography (CT) were performed to localize the abnormality prior to PET-CT. All the patients underwent CT and PET-CT in the period from January 2008 until January 2012. All PET images were interpreted in conjunction with thoracic CT. PET-CT and CT diagnoses were correlated with pathological diagnoses. RESULTS: Forty-five patients had recurrent tumour. Tumour recurrence was observed more often in men than in women and also in case of neoplastic cell emboli in lymphatic or blood vessels. In three patients second primary lung cancer was diagnosed. False positive diagnosis of relapse based on PET-CT was obtained in 4 patients, mainly due to inflammatory lesions. The accuracy of PET-CT for diagnosis of recurrence was 94.4% (95% CI 91; 100). CONCLUSIONS: FDG PET-CT was the best method to differentiate recurrent bronchogenic carcinoma from inflammatory lesions, especially at post-therapeutic sites. It has been shown that PET-CT is more accurate method than CT in recurrent NSCLC. PET-CT results had a further impact on the clinical management and treatment planning.

[Autoimmunological pulmonary alveolar proteinosis--case report]. / Autoimmunologiczna proteinoza pecherzyków plucnych--opis przypadku.

Autoimmunological pulmonary alveolar proteinosis (APAP) is a rare interstitial lung disease with abnormal surfactant homeostasis. Autoimmunological pulmonary alveolar proteinosis is diagnosed most often in the third or fourth decade of life. Predominant symptoms are dyspnea and cough. In most cases, disease is mild but in more severe cases when dyspnea limits patient physical activity a treatment is required. The most common treatment procedure is a whole-lung lavage. We present a case study of 37 years old woman with the patchy consolidations in the chest radiograph. High resolution computed tomography (HRCT) image suggested hipersensivity pneumonitis. At the beginning due to limited disease symptoms no specific proceedings was implemented. After two year follow-up of non-resolving pulmonary changes the decision about open lung biopsy was made. On the basis of histological examination of samples and presence of anty GM-CSF antibodies the diagnosis of autoimmunological pulmonary alveolar proteinosis was established.

Asymptomatic appearance of splenic infarction in Wegener's granulomatosis.

Splenic involvements in Wegener's granulomatosis (WG) are rarely diagnosed ante-mortem, while an autopsy is able to reveal a high rate of spleen lesions (78-100%). To date, there have been a few reported cases of splenic abnormalities in WG, including: splenomegaly, capsular adhesion, dysfunction and infarction. We reported a case of biopsy-verified WG with radiological evidence of diffuse spleen infarction despite the lack of any clinical symptoms. We concluded that due to a potential risk of severe hemorrhagic complications when anticoagulant therapy is necessary, radiological assessment of spleen should be performed regularly in this group of patients, particularly because spleen involvement can be asymptomatic.