Atypical manifestations of granulomatosis with polyangiitis: the diagnostic challenge for pulmonologists

This is a review considering atypical manifestations of granulomatosis with polyangiitis (GPA). Virtually any organ can be affected, and in some patients, GPA can manifest unusually. Since thoracic involvement of GPA often predominates, the first who might be expected to establish a diagnosis are pulmonary specialists. We would like to familiarize pulmonary specialists with several extra-ELK (E: ear-nose-throat; L: lung; K: kidney) involvements of the disease. We describe sites rarely affected by GPA like the breast, skeletal system, orbit and eye, heart and vessels, central nervous system, urogenital system as well as endocrine and gastrointestinal tract involvement

IPF Respiratory Symptoms Management — Current Evidence

Idiopathic pulmonary fibrosis (IPF) is a progressive, chronic disease of the lungs which is characterized by heavy symptom burden, especially in the last year of life. Despite recently established anti-fibrotic treatment IPF prognosis is one of the worst among interstitial lung diseases. In this review available evidence regarding pharmacological and non-pharmacological management of the main IPF symptoms, dyspnea and cough, is presented

Smoking and lung cancer

W pracy omówiono związek między paleniem tytoniu a rakiem płuca. Scharakteryzowano molekularne zaburzenia powstające pod wpływem czynników rakotwórczych zawartych w dymie tytoniowym oraz konstytutywne profile genetyczne zwiększające ryzyko zachorowania na ten nowotwór. Przedstawiono potencjalne możliwości zastosowania testów molekularnych w wyodrębnianiu grup szczególnego ryzyka wśród nałogowych palaczy papierosów. Podkreślono także korzystny wpływ zerwania z nałogiem palenia tytoniu u chorych na niedrobnokomórkowego raka płuca, w tym także po zakończeniu radykalnego leczenia.In this review we presented a relation between cigarette smoking and lung cancer. We characterized molecular alterations resulting from carcinogens present in the cigarette smoke and presented the constitutive genetic profiles related to the increased risk of lung cancer. We also discussed a possible use of these profiles in the selection of high risk groups among the heavy smokers. Finally, a positive impact of quitting smoking in lung cancer patients, including those who have undergone curative resection, was presented

How do relatives remember the dying of lung cancer patients after 2-8 years? A qualitative study based on in-depth interviews with family members

Background. The aim of our study was to assess how relatives remember the dying of lung cancer patients several years after their death. Methods. The family members of 45 non small-cell lung cancer patients who died up to 7 years after radical surgical treatment were telephoned and asked to meet the research team. Only 20 of them agreed to talk about the dying and death of their relatives; for the rest the issue was still too painful. During the meeting with 2 researchers, participants were asked to comment freely on the points of the Assessment of Dying in Lung Cancer Patients questionnaire and to score the Quality of Dying Apgar. Results. Family members of 15 of the patients stated recognizing the dying of their loved one. They concluded this on the basis of exacerbation of symptoms, "typical" appearance, change in behaviour or particular words the patient said to them. The median score of the quality of dying was 6.5/10 (range 3-9). Lower scoring was observed for patients who died in hospital, without the family present at the moment of death and with uncontrolled symptoms. Only 5 of the relatives assessed pain and other symptom control as satisfactory. Other family members remembered pain (n = 9), dyspnoea (n = 8), delirium (n = 4) and other symptoms as disturbing the dying in a severe or moderate way. Three relatives assessed that their loved one's dying was not "dignified", mostly due to the severe physical suffering, the nursing being crucially remiss at the hospital and the patient's being alone at the moment of death. All family members were able to describe the healthcare professionals' attitudes and even repeat the statements they heard from them. Conclusion. We have demonstrated that several years after death many of the words and the behaviour of the dying patients were still alive in their relatives' memories. The family members remember how the healthcare professionals behaved and what they said to them. While caring for dying people, we should bear in mind that the way in which we behave may either give strength to the family or destroy their peace of mind for many years.Background. The aim of our study was to assess how relatives remember the dying of lung cancer patients several years after their death. Methods. The family members of 45 non small-cell lung cancer patients who died up to 7 years after radical surgical treatment were telephoned and asked to meet the research team. Only 20 of them agreed to talk about the dying and death of their relatives; for the rest the issue was still too painful. During the meeting with 2 researchers, participants were asked to comment freely on the points of the Assessment of Dying in Lung Cancer Patients questionnaire and to score the Quality of Dying Apgar. Results. Family members of 15 of the patients stated recognizing the dying of their loved one. They concluded this on the basis of exacerbation of symptoms, "typical" appearance, change in behaviour or particular words the patient said to them. The median score of the quality of dying was 6.5/10 (range 3-9). Lower scoring was observed for patients who died in hospital, without the family present at the moment of death and with uncontrolled symptoms. Only 5 of the relatives assessed pain and other symptom control as satisfactory. Other family members remembered pain (n = 9), dyspnoea (n = 8), delirium (n = 4) and other symptoms as disturbing the dying in a severe or moderate way. Three relatives assessed that their loved one's dying was not "dignified", mostly due to the severe physical suffering, the nursing being crucially remiss at the hospital and the patient's being alone at the moment of death. All family members were able to describe the healthcare professionals' attitudes and even repeat the statements they heard from them. Conclusion. We have demonstrated that several years after death many of the words and the behaviour of the dying patients were still alive in their relatives' memories. The family members remember how the healthcare professionals behaved and what they said to them. While caring for dying people, we should bear in mind that the way in which we behave may either give strength to the family or destroy their peace of mind for many years

The last months of lung cancer patients’ lives, in the memory of their relatives. A qualitative study based on in-depth interviews with family members

Background. The aim of the study was to assess how family members remember the final months of their loved ones 2–8 years after their death. We focused particularly on their recognition of the patients’ physical, psychosocial and spiritual needs. Methods. The family members of 45 non small-cell lung cancer patients who died up to 7 years after radical surgical treatment were telephoned and asked to meet the research team. Only 20 of them agreed to talk about the dying and death of their relatives; for the rest the issue was still too painful. During the meeting with two researchers, participants were asked to comment freely on the points of the Assessment of Dying in Lung Cancer Patients questionnaire. Results. Almost all the relatives interviewed in our study were able to recognize the beginning of the terminal phase. They connected the beginning of dying with the deterioration of the physical and/or psychological status of patients, such as the exacerbation of weakness and/or other symptoms or with clearly distinguished incidents such as cancer recurrence or hip fracture. The majority were able to define the length of this phase as being several months (median = 3, range 1–11 months). The most common physical symptoms mentioned by the relatives interviewed were, in sequential order: pain (n = 13) and fatigue (n = 13), anorexia (n = 9), dyspnoea (n = 7) and cachexia (n = 7). Of the 20 decedents, 18 regularly received painkillers at least at some period during their final months. Apart from medicines prescribed by the doctors, 9 patients were treated with “anticancer” herbs, or homeopathy, or by bioenergotherapy. From the relatives’ perspectives, the main approach was focused on the physical aspects of care while there was a lack of psychological and social support, the latter often causing severe financial burden. Most relatives believed that chaplains are the main source of spiritual comfort and there was easy access to such a service both in their parish and in the hospice or hospitals. Conclusion. Our study showed that the families remembered feelings of loneliness and helplessness when confronted with the psychological suffering of their loved ones and the financial burdens caused by the caregivers. Professionals involved in palliative care should acknowledge that holistic care requires sensitivity, not only to the physical but also to the psychosocial and spiritual aspects of end-of-life care.Background. The aim of the study was to assess how family members remember the final months of their loved ones 2–8 years after their death. We focused particularly on their recognition of the patients’ physical, psychosocial and spiritual needs. Methods. The family members of 45 non small-cell lung cancer patients who died up to 7 years after radical surgical treatment were telephoned and asked to meet the research team. Only 20 of them agreed to talk about the dying and death of their relatives; for the rest the issue was still too painful. During the meeting with two researchers, participants were asked to comment freely on the points of the Assessment of Dying in Lung Cancer Patients questionnaire. Results. Almost all the relatives interviewed in our study were able to recognize the beginning of the terminal phase. They connected the beginning of dying with the deterioration of the physical and/or psychological status of patients, such as the exacerbation of weakness and/or other symptoms or with clearly distinguished incidents such as cancer recurrence or hip fracture. The majority were able to define the length of this phase as being several months (median = 3, range 1–11 months). The most common physical symptoms mentioned by the relatives interviewed were, in sequential order: pain (n = 13) and fatigue (n = 13), anorexia (n = 9), dyspnoea (n = 7) and cachexia (n = 7). Of the 20 decedents, 18 regularly received painkillers at least at some period during their final months. Apart from medicines prescribed by the doctors, 9 patients were treated with “anticancer” herbs, or homeopathy, or by bioenergotherapy. From the relatives’ perspectives, the main approach was focused on the physical aspects of care while there was a lack of psychological and social support, the latter often causing severe financial burden. Most relatives believed that chaplains are the main source of spiritual comfort and there was easy access to such a service both in their parish and in the hospice or hospitals. Conclusion. Our study showed that the families remembered feelings of loneliness and helplessness when confronted with the psychological suffering of their loved ones and the financial burdens caused by the caregivers. Professionals involved in palliative care should acknowledge that holistic care requires sensitivity, not only to the physical but also to the psychosocial and spiritual aspects of end-of-life care

Choroba śródmiąższowa płuc w przebiegu układowych chorób tkanki łącznej — wspólny problem reumatologa i pulmonologa

Choroba śródmiąższowa płuc jest częstym i niebezpiecznym powikłaniem wielu chorób reumatologicznych. Dzięki postępowi, jaki dokonał się w ciągu ostatnich lat, wzrosły możliwości diagnostyczne, pojawiły się nowe opcje terapeutyczne, a przede wszystkim pogłębiło się zrozumienie wagi tego problemu. Choroby śródmiąższowe stanowią niejednorodną grupę, którą różnicuje się przy użyciu badań histopatologicznych oraz obrazowych. Rokowanie i zalecana terapia zależy nie tylko od postaci choroby śródmiąższowej, ale w znacznej mierze od choroby reumatologicznej leżącej u jej podłoża. Celem tego artykułu jest przedstawienie patologii, jaką jest choroba śródmiąższowa płuc, oraz aktualnej wiedzy na temat jej leczenia. Słowa kluczowe: choroba śródmiąższowa płuc, choroba tkanki łącznej, diagnostyka, terapi

Jak najbliżsi pamiętają umieranie chorych na raka płuca po 2-8 latach? Badanie jakościowe oparte na wywiadach z członkami rodzin

Wstęp. Celem badania była ocena, jak bliscy pamiętają umieranie chorych na raka płuca kilka lat po ich śmierci. Materiał i metody. Zaproszenie do udziału w badaniu przekazano telefonicznie rodzinom 45 chorych, którzy zmarli do 7 lat po radykalnej resekcji niedrobnokomórkowego raka płuca. Tylko 20 z zaproszonych osób zgodziło się rozmawiać o umieraniu i śmierci ich najbliższych. Dla pozostałych ten temat był ciągle zbyt bolesny. Podczas spotkania z dwoma badaczami uczestników poproszono, aby w swobodny sposób odnieśli się do problemów poruszonych w kwestionariuszu Oceny Umierania Chorych na Raka Płuca oraz spróbowali okreslić jakość umierania według skali The Quality of Dying Apgar (QODA). Wyniki. Krewni 15 pacjentów uważali, że byli w stanie rozpoznać okres umierania ich bliskich na podstawie nasilenia objawów, „typowego” wyglądu, zmiany zachowania lub szczególnych słów, jakie od nich usłyszeli. Mediana punktacji w skali oceniającej jakość umierania według QODA wynosiła 6,5/10 (przedział 3-9). Niższe oceny zanotowano w przypadku pacjentów umierających w szpitalu, których rodziny były nieobecne w czasie zgonu, a także jeśli objawy leczono w niedostateczny sposób. Jedynie rodziny 5 pacjentów uważały łagodzenie bólu i innych objawów w ostatnich dniach życia za zadowalające. Pozostali pamiętali, że ból (n = 9), duszność (n = 8), splątanie (n = 4) lub inne objawy zaburzały umieranie w znacznym lub bardzo nasilonym stopniu. Krewni 3 chorych stwierdzili, że umieranie ich bliskich nie było „pełne godności” przede wszystkim z powodu bardzo nasilonych objawów fizycznych, drastycznych zaniedbań pielęgnacyjnych w szpitalu i poczucia opuszczenia w momencie zgonu. Wszyscy uczestnicy badania byli w stanie opisać postawę lekarzy i pielęgniarek, a nawet powtórzyć wypowiedzi, które od nich usłyszeli. Wnioski. W badaniu pokazano, że nawet po kilku latach wiele słów, gestów i zachowań chorych z okresu ich umierania przechowywanych jest w pamięci ich rodzin. Członkowie rodzin pamiętają też, jak zachowywał się personel medyczny i jakich słów używał. Opiekując się umierającymi, należy uwzględnić, że sposób, w jaki się to robi, ma dla ich bliskich duże znaczenie i może ich albo wzmacniać, albo osłabiać na długie lata

Quality of life of patients with asthma which has been well and poorly controlled

Wstęp: Celem niniejszej pracy była ocena jakości życia chorych na astmę oskrzelową, dobrze i źle kontrolowaną, w zależności od stopnia kontroli choroby i jej zaawansowania. Materiał i metody: Do badania włączono 70 chorych z rozpoznaną astmą, w wieku 18-40 lat. Badanie trwało od listopada 2005 do lutego 2006 roku w Szpitalu Specjalistycznym w Chojnicach i w Przyklinicznej Poradni Alergologicznej Kliniki Alergologii Akademii Medycznej w Gdańsku. Rozpoznanie, zaawansowanie oraz stopień kontroli astmy były określane przez lekarza. Jakość życia chorych oceniono na podstawie kwestionariusza Szpitala Świętego Jerzego (SGRQ). Analiza statystyczna przeprowadzona była przy użyciu komputerowego programu Statistica. Badanie uzyskało akceptację lokalnej Komisji Etycznej. Wyniki: Zgodnie z zaleceniami GINA u 26 chorych rozpoznano łagodną astmę, u 31 - umiarkowaną, a u 13 - ciężką. U 41 pacjentów kontrola objawów była dobra, a u 29 - zła. Starszy wiek chorych oraz czas trwania schorzenia były istotnie związane z ciężką postacią choroby, odpowiednio: p = 0,01 i p = 0,003. W grupie chorych z dobrą kontrolą objawów całkowita suma punktów testu SGRQ wyniosła 38,77 i była znamiennie niższa niż w grupie chorych ze złą kontrolą objawów - 54,2; p = 0,0001. Podobnie istotne różnice stwierdzono we wszystkich domenach testu (objawy, aktywność i wpływ choroby na życie pacjentów) - odpowiednio: p = 0,014; p = 0,035; p = 0,003. Wnioski: Jakość życia pacjentów w istotnym stopniu zależy od kontroli objawów choroby.Introduction: The aim of the study was to assess the quality of life of patients with asthma who were well and poorly controlled. Material and methods: 70 patients with diagnosed asthma, aged from 18 to 40, were included into the study between November 2005 and February 2006 at the Specialist Hospital in Chojnice and Allergy out-patient Clinic of Medical University of Gdańsk. The diagnosis and stage of asthma, as well as the assessment of the control of disease was performed by the physician. Quality of life was assessed with the use of St. George Respiratory Questionnaire (SGRQ). Statistical analysis was made with the use of computer statistical program Statistica. Results: According to GINA in 26 patients mild asthma was diagnosed, in 31 - moderated and in 13 - severe disease. 41 patients were assessed as well controlled and 29 - as poorly controlled. Older age and longer duration of the disease were related to severe asthma, p = 0.01 and p = 0.003, respectively. In well controlled patients overall score for SGRQ was 48.86, whereas in poorly controlled was - 74.4. There was a significant difference between the overall quality of life in well and poorly controlled patients with asthma, p = 0.0001. This difference was found in all three domains: for symptoms, activity and impact on life, p = 0.014, p = 0.035 and p = 0.003, respectively. Conclusions: Quality of life is strongly dependent on the control of symptoms in asthmatic patients

BRCA1: A Novel Prognostic Factor in Resected Non-Small-Cell Lung Cancer

BACKGROUND: Although early-stage non-small-cell lung cancer (NSCLC) is considered a potentially curable disease following complete resection, patients have a wide spectrum of survival according to stage (IB, II, IIIA). Within each stage, gene expression profiles can identify patients with a higher risk of recurrence. We hypothesized that altered mRNA expression in nine genes could help to predict disease outcome: excision repair cross-complementing 1 (ERCC1), myeloid zinc finger 1 (MZF1) and Twist1 (which regulate N-cadherin expression), ribonucleotide reductase subunit M1 (RRM1), thioredoxin-1 (TRX1), tyrosyl-DNA phosphodiesterase (Tdp1), nuclear factor of activated T cells (NFAT), BRCA1, and the human homolog of yeast budding uninhibited by benzimidazole (BubR1). METHODOLOGY AND PRINCIPAL FINDINGS: We performed real-time quantitative polymerase chain reaction (RT-QPCR) in frozen lung cancer tissue specimens from 126 chemonaive NSCLC patients who had undergone surgical resection and evaluated the association between gene expression levels and survival. For validation, we used paraffin-embedded specimens from 58 other NSCLC patients. A strong inter-gene correlation was observed between expression levels of all genes except NFAT. A Cox proportional hazards model indicated that along with disease stage, BRCA1 mRNA expression significantly correlated with overall survival (hazard ratio [HR], 1.98 [95% confidence interval (CI), 1.11-6]; P = 0.02). In the independent cohort of 58 patients, BRCA1 mRNA expression also significantly correlated with survival (HR, 2.4 [95%CI, 1.01-5.92]; P = 0.04). CONCLUSIONS: Overexpression of BRCA1 mRNA was strongly associated with poor survival in NSCLC patients, and the validation of this finding in an independent data set further strengthened this association. Since BRCA1 mRNA expression has previously been linked to differential sensitivity to cisplatin and antimicrotubule drugs, BRCA1 mRNA expression may provide additional information for customizing adjuvant antimicrotubule-based chemotherapy, especially in stage IB, where the role of adjuvant chemotherapy has not been clearly demonstrated