Pulmonary hypertension in diffuse parenchymal lung diseases — is there any benefit of PAH-specific therapy?

  Pulmonary hypertension (PH) is diagnosed in 40–50% of the patients with end-stage diffuse parenchymal lung diseases (DPLD), and it is associated with significant worsening of life expectancy. Latest ERS/ESC guidelines recommend best available treatment of DPLD, and long-term oxygen therapy in the patients with PaO2 less than 60 mm Hg. Pulmonary arterial hypertension (PAH)-targeted drugs are not recommended in PH-DPLD patients, due to the risk of increasing the ventilation-perfusion mismatch, and consequently worsening of hypoxaemia. Nevertheless, PAH-oriented treatment may be beneficial to selected groups of patients. The authors try to find the answer to several important questions: is there any benefit of PAH-specific therapy in PH-DPLD, who should be the candidate for PAH-specific therapy, what class of drugs is most promising, and what outcome measures should be employed?

Respiratory system involvement in inflammatory bowel diseases

  Inflammatory bowel diseases are systemic disorders that can manifest in any location. The problem of respiratory system involvement is very important form clinical point of view. In the article we try to systematize the current knowledge on this topic.

Antiphospholipid syndrome - an update on diagnosis and treatment

Antiphospholipid syndrome (APS) was described by Hughes et al in 1986 and then by Harris et al in 1987. The symptoms of disease are related to the presence of autoantibodies directed against plasma and serum proteins that form complexes with phospholipids of cell membranes. The immunologic process involving vascular endothelium, thrombocytes and coagulation factors is responsible for enhancement of procoagulation mechanisms and as a result - venous and arterial thrombosis. Actual criteria of recognition of APS were published in 2006 as a result of 11th International Congress on Antiphospholipid Antibodies which was taking place in 2004 in Sydney. Multiorgan symptoms of disease, criteria of recognition and methods of treatment were discussed in the present paper.Zespół antyfosfolipidowy (APS) został opisany po raz pierwszy przez Hughesa i wsp. w 1986 roku, a następnie przez Harrisa i wsp. w 1987 roku. Objawy choroby związane są z obecnością autoprzeciwciał skierowanych przeciwko białkom tworzącym kompleksy z fosfolipidami błon komórkowych. Toczący się proces immunologiczny zmienia właściwości śródbłonka naczyń krwionośnych, płytek krwi i białek układu krzepnięcia, prowadząc do rozwoju zakrzepicy w naczyniach tętniczych i żylnych. Definicja APS ulegała wielokrotnym modyfikacjom, aktualnie obowiązują kryteria rozpoznawania ustalone w 2004 roku w Sydney podczas 11. Międzynarodowego Kongresu dotyczącego Przeciwciał Antyfosfolipidowych, a opublikowane w 2006 roku. W pracy omówiono wielonarządowe objawy choroby, zasady jej rozpoznawania i leczenia

Secretion of beta-human chorionic gonadotropin by non-small cell lung cancer: a case report

<p>Abstract</p> <p>Introduction</p> <p>We describe a case of non-small cell lung cancer that was found to stain positive for beta-human chorionic gonadotropin on immunohistochemistry. Only a few case reports have described lung cancers that secrete beta-human chorionic gonadotropin.</p> <p>Case presentation</p> <p>A 68-year-old Caucasian man presented with symptoms of weakness, fatigue and weight loss for the past two months. On examination, he was found to have generalized lymphadenopathy, and radiologic workup revealed numerous metastases in the lungs, liver and kidneys. Biopsy of the supraclavicular lymph node revealed metastatic large cell lung cancer with beta-human chorionic gonadotropin hormone positivity. The serum beta-human chorionic gonadotropin level was 11,286 mIU/ml (upper limit of normal, 0.5 mIU/ml in non-pregnant females). He was diagnosed with stage 4 lung non-small cell lung cancer. The patient refused chemotherapy. He was discharged home with hospice care.</p> <p>Conclusion</p> <p>The markedly elevated serum values of beta-human chorionic gonadotropin initially prompted the medical team to investigate germinal tumors. In the presence of a negative testicular ultrasound, workup was performed to find an extratesticular source of the tumor. Finally, the diagnosis was made with a tissue biopsy. This case illustrates that atypical markers can be seen in many cancers, emphasizing the role of immunohistochemistry and tissue biopsy in establishing the diagnosis.</p

Pulmonary hypertension in the course of diffuse parenchymal lung diseases — state of art and future considerations

Lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. According to the most recent publications, PH in the course of lung diseases develops as a result of both “parenchymal” and vascular pathology, in the patients with genetic predisposition. Prolonged infection (especially viral one) may be an additional promoting factor. Right heart catheterization (RHC), which is an invasive procedure, is the only objective method of diagnosing PH. According to the latest recommendations, the management algorithm of PH and coexisting interstitial lung disease is based on RHC and the results of pulmonary function tests. Majority of the patients develop mild PH in the course of advanced lung disease. Best treatment of underlying lung pathology combined with long term oxygen treatment is recommended in this group. In case of severe PH (mean resting pulmonary artery pressure (mPAP) ≥ 35 mm Hg) the alternate cause of PH has to be sought. PAH-specific drugs use should be limited to patients with severe PH participating in clinical trials. In this review, the value of various non-invasive methods (echocardiography, radiological examination, exercise capacity and brain natriuretic peptides assessment) in the process of screening for PH is presented, and the results of recent randomized clinical trials with PAH-specific drugs in patients with diffuse parenchymal lung diseases are discussed.Lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. According to the most recent publications, PH in the course of lung diseases develops as a result of both “parenchymal” and vascular pathology, in the patients with genetic predisposition. Prolonged infection (especially viral one) may be an additional promoting factor. Right heart catheterization (RHC), which is an invasive procedure, is the only objective method of diagnosing PH. According to the latest recommendations, the management algorithm of PH and coexisting interstitial lung disease is based on RHC and the results of pulmonary function tests. Majority of the patients develop mild PH in the course of advanced lung disease. Best treatment of underlying lung pathology combined with long term oxygen treatment is recommended in this group. In case of severe PH (mean resting pulmonary artery pressure (mPAP) ≥ 35 mm Hg) the alternate cause of PH has to be sought. PAH-specific drugs use should be limited to patients with severe PH participating in clinical trials. In this review, the value of various non-invasive methods (echocardiography, radiological examination, exercise capacity and brain natriuretic peptides assessment) in the process of screening for PH is presented, and the results of recent randomized clinical trials with PAH-specific drugs in patients with diffuse parenchymal lung diseases are discussed

Lung mycobacteriosis - clinical presentation, diagnostics and treatment

Prątki niegruźlicze to grupa bakterii, które wywołują u ludzi chorobę zwaną mikobakteriozą, w odróżnieniu od prątków powodujących gruźlicę lub trąd. Prątki niegruźlicze występują powszechnie w środowisku, są izolowane z wody, powietrza, gleby. Do zakażenia dochodzi drogą inhalacyjną, pokarmową i przez uszkodzony naskórek. Prątki niegruźlicze charakteryzują się niską patogennością; chorują zwykle osoby z upośledzoną odpornością oraz osoby z uprzednio występującą chorobą płuc. Nie ma dowodów na przenoszenie zakażenia pomiędzy ludźmi. Najczęstszą postacią kliniczną mikobakteriozy jest choroba płuc. Diagnostyka oparta na metodach molekularnych pozwala na szybszą identyfikację gatunków prątków niegruźliczych i odróżnienie ich od prątków gruźliczych. W pracy przedstawiono współczesne poglądy na temat epidemiologii, obrazu klinicznego i radiologicznego oraz sposobu rozpoznawania i metod leczenia płucnych postaci mikobakteriozy. Pneumonol. Alergol. Pol. 2010; 78, 2: 138-147Nontuberculous mycobacteria (NTM) are a group of bacteria that may cause human disease mycobacteriosis, but do not cause tuberculosis or leprosy. NTM are acquired through environmental exposure to water, aerosols, soil, dust and are transfered to human through inhalation, ingestion, and skin lesions, due to injuries, surgical procedures, or intravenous catheters. People with suppressed immune response, with pre-existing lung damage in the course of various lung diseases are most likely to be affected. There is no evidence of person-to-person spread of these diseases. A variety of manifestations of NTM infection have been described, but the lungs remain the most commonly involved site. Molecular methods allow the quicker differentiation of NTM from TB isolates and help to identify new NTM species. The purpose of this article is to review the common clinical manifestations of NTM lung disease, the conditions associated with NTM lung disease, diagnostic criteria and treatment of the most frequent species of NTM. Pneumonol. Alergol. Pol. 2010; 78, 2: 138-14

What factors may influence epidemiological situation of tuberculosis in Poland and in the world?

The authors present the review of factors influencing epidemiological situation of tuberculosis in Poland and in the world. The groups of increased risk of tuberculosis, and clinical conditions predisposing to activation of latent tuberculosis infection (LTBI) such as HIV, uremia, diabetes mellitus, transplantation of organs, treatment with glucocorticosteroids and with antibodies to TNF and to its receptors, were presented. The higher prevalence and worse prognosis of tuberculosis in elderly people was emphasised. The methods of LTBI recognition, according to recent recommendations, with special consideration to patients in immunosupression, were shown. Methods of treatment to prevent LTBI activation, according to WHO experts, were also presented. All data were discussed in relation to the actual epidemiological situation of tuberculosis in Poland.The authors present the review of factors influencing epidemiological situation of tuberculosis in Poland and in the world. The groups of increased risk of tuberculosis, and clinical conditions predisposing to activation of latent tuberculosis infection (LTBI) such as HIV, uremia, diabetes mellitus, transplantation of organs, treatment with glucocorticosteroids and with antibodies to TNF and to its receptors, were presented. The higher prevalence and worse prognosis of tuberculosis in elderly people was emphasised. The methods of LTBI recognition, according to recent recommendations, with special consideration to patients in immunosupression, were shown. Methods of treatment to prevent LTBI activation, according to WHO experts, were also presented. All data were discussed in relation to the actual epidemiological situation of tuberculosis in Poland