Publications by authors named "Justyna FijoLek"
Background: Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.
Aim: To characterize chILD-survivors and identify their impact on adult-ILD centers.
Granulomatosis with polyangiitis: clinical characteristics and updates in diagnosis.
Front Med (Lausanne)
August 2024
Article Synopsis
- Granulomatosis with polyangiitis (GPA) is a rare disease causing inflammation in the respiratory system and blood vessel damage, often linked to specific antibodies (ANCA) against certain proteins in white blood cells.
- * Diagnosis involves clinical evaluation, imaging, and blood tests, but biopsies, while recommended, are not always practical and may not show clear findings.
- * Advances in understanding the disease’s characteristics have improved patient classification and treatment strategies, but there is still no reliable method to predict disease activity or relapses.
Background: Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous inflammation with necrotizing vasculitis predominantly affecting small to medium vessels. The survival rates have drastically improved; however, GPA can be lethal, with older patients having a worse prognosis and higher mortality than younger patients. Moreover, the incidence of various cancers has been reported to increase in patients with GPA.
View Article and Find Full Text PDFEosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease characterized by eosinophil-rich granulomatous inflammation and necrotizing vasculitis, pre-dominantly affecting small-to-medium-sized vessels. It is categorized as a primary antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) but also shares features of hypereosinophilic syndrome (HES); therefore, both vessel inflammation and eosinophilic infiltration are suggested to cause organ damage. This dual nature of the disease causes variable clinical presentation.
View Article and Find Full Text PDFCryptogenic organizing pneumonia (COP) is a form of idiopathic interstitial pneumonia that results from the pulmonary reaction to various unidentified injuries. Secondary organizing pneumonia is diagnosed when the triggering factor has been identified; it is mainly caused by infections, toxic substance exposure, drugs, connective tissue diseases, malignancies, autoimmune diseases, bone marrow, or organ transplantation, and radiotherapy. There has been an increase in the number of reports of drug-induced organizing pneumonia (OP).
View Article and Find Full Text PDFArticle Synopsis
- The study aimed to identify risk factors for relapse and death in patients with eosinophilic granulomatosis with polyangiitis (EGPA), particularly those negative for antineutrophil cytoplasmic antibodies.
- Among 86 patients analyzed, nearly 35% experienced relapses, and about 9% died; certain factors like severe EGPA and cardiac issues increased death risk, while prolonged corticosteroid treatments raised relapse risk.
- Overall, relapses are common but do not affect mortality, and while cardiac involvement is frequent, chronic asthma treatment correlates with lower death risk despite higher relapse rates.
Article Synopsis
- Granulomatosis with polyangiitis (GPA) is a rare condition that primarily targets the upper respiratory system, lungs, and kidneys, though other organs may be affected less frequently.
- Cardiac involvement in GPA is uncommon, and this case highlights a unique instance where thoracic pain was the only symptom presented.
- The diagnosis was achieved through a comprehensive approach utilizing various imaging techniques and cardiac histology to identify the underlying issue.
Article Synopsis
- Eosinophilia, a condition characterized by high levels of eosinophils in the blood, is significant for diagnosing lung disorders and has important implications for treatment and prognosis.
- The study included 46 patients with eosinophilia and respiratory issues, using standard diagnostic methods and various statistical analyses to explore the relationship between eosinophil levels and clinical symptoms.
- Results indicated that severe eosinophilia (≥5,000 cells/μl) was linked to more severe health problems, including skin, heart, and kidney diseases, and higher incidence of vasculitis compared to those with milder forms of eosinophilia.
Article Synopsis
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare group of diseases that cause inflammation in small blood vessels, often linked to the presence of ANCA.
- Recent research highlights the role of neutrophil extracellular traps (NETs) in AAV, suggesting they may contribute to ANCA formation.
- While ANCA serology is useful for classifying AAV and understanding its various forms, there is debate over the effectiveness of using serial ANCA measurements to track treatment and predict relapses.
Article Synopsis
- - Granulomatosis with polyangiitis (GPA) is a rare inflammatory disease that primarily affects the respiratory system and involves inflammation of small to medium blood vessels.
- - Diagnosis can be challenging due to vague symptoms and varying clinical presentations, often leading to misdiagnosis, especially in older patients.
- - The report highlights two elderly cases where GPA was only identified 4 to 6 years after the initial symptoms, despite multiple diagnostic procedures being performed.
Objective: Antineutrophil cytoplasmic antibodies (ANCAs) are considered a risk factor for granulomatosis with polyangiitis (GPA) exacerbation, especially when staphylococcal superantigens (SAgs) are present in nasal swabs. Their role in monitoring disease activity remains controversial. This study determined the relationship of ANCAs with disease activity and presence of SAgs in GPA patients.
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- The study explored the relationship between nasal carriage of Staphylococcus aureus and superantigens (SAg) with disease activity in patients with granulomatosis with polyangiitis (GPA), involving 150 hospitalized patients from 2009-2016.
- Results showed SAg present in about 35% of nasal swabs but did not establish a significant correlation with GPA activity, although specific SAg like SED and TSST-1 appeared more frequently in active cases.
- The use of trimethoprim/sulfamethoxazole treatment was found to be protective, while limited disease (subglottic stenosis) was linked to increased activity, indicating further mechanisms are needed to understand GPA exacerbation
Article Synopsis
- Clinicians often find it difficult to differentiate between pulmonary tuberculosis and lung cancer, as the symptoms and imaging results can be quite similar.
- A case report describes a patient who had been treated for advanced lung cancer a decade ago and was recently hospitalized due to concerns about cancer recurrence.
- Ultimately, despite the initial suspicion of cancer progression, the patient was diagnosed with tuberculosis.
Article Synopsis
- Diffuse alveolar haemorrhage (DAH) is a serious condition caused by damage to the small blood vessels in the lungs, leading to the accumulation of red blood cells in the air spaces.
- The prognosis and treatment for DAH depend on the underlying disease, and it can complicate conditions like venous thromboembolism (VTE), making management difficult for healthcare providers.
- A case study is presented of a young patient who experienced severe DAH due to anti-GBM disease, which was further complicated by a pulmonary embolism.
Article Synopsis
- - Sarcoidosis is a disease primarily impacting the lungs, often resolving on its own, but treatment is necessary for severe cases involving critical organs or progressive disease.
- - A study analyzed data from 1,810 sarcoidosis patients discharged between 2010 and 2013, revealing that only 15.5% received systemic immune-modulating treatment, with men and patients over 50 being the most likely to be treated.
- - The findings indicate that less than 16% of sarcoidosis patients generally need systemic treatment, with those experiencing involvement of organs other than the lungs being significantly more likely to require intervention.
Article Synopsis
- * Out of 250 GPA patients, 34 (13.6%) developed SGS, and this condition often appeared independently of other disease symptoms, particularly during or after immunosuppressive therapy.
- * IDIT was performed on all patients, leading to immediate improvement, with a median response time of 37 months and no need for tracheostomy, suggesting IDIT is a reliable treatment option for patients with SGS related to GPA.
Background: Cardiac magnetic resonance imaging (CMRI) has emerged as a sensitive and non-invasive technique in the evaluation of cardiac lesions in eosinophilic granulomatosis with polyangiitis (EGPA) patients.
Objectives: To evaluate the ability of CMRI to detection and monitoring of the treatment efficacy in EGPA patients with cardiac involvement.
Methods: To the retrospective-prospective study were enrolled 33 cardiac involvement EGPA patients.
Pulmonary alveolar proteinosis is a very rare interstitial lung disease caused by abnormal intra-alveolar surfactant accumulation. Usually, it appears as a "crazy-paving" pattern on high-resolution computed tomography. The image is so typical, that together with the characteristic bronchoalveolar lavage examination with presence of Periodic Acid Schiff positive substance is sufficient for establishing diagnosis, without histological confirmation.
View Article and Find Full Text PDFPulmonary alveolar proteinosis during a 30-year observation. Diagnosis and treatment.
Pneumonol Alergol Pol
September 2016
Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the abnormal accumulation of surfactant-like material in macrophages within the alveolar spaces and distal bronchioles. The course of the disease is variable and the prognosis is often good. However, progressive disease in some patients can cause respiratory dysfunction and can be life threatening.
View Article and Find Full Text PDFThe antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies and the association of protean clinical manifestations as a result of both venous and arterial thrombosis. While pulmonary embolism (secondary to deep vein thrombosis) is common and well-known disturbance in antiphospholipid syndrome, recently there are growing number of case reports describing nonthrombotic lung pathologies in APS. We present here a young male with antiphospholipid syndrome, whose the only manifestation was diffuse alveolar hemorrhage.
View Article and Find Full Text PDFIntroduction: Inherited alpha-1 antitrypsin (AAT) deficiency is one of the three most common genetic disorders in Caucasians. It considerably increases the risk of progressive obstructive lung diseases, mostly chronic obstructive pulmonary disease. It has also been suggested that AAT deficiency might be instrumental vasculitis associated with the anti-neutrophil cytoplasm antibodies (cANCA) and subsequent lung tissue injury.
View Article and Find Full Text PDF[Yellow nail syndrome in a patient with membranous glomerulonephritis].
Pneumonol Alergol Pol
May 2012
Yellow nail syndrome (YNS) is a condition characterized by yellow-green coloration of nails, respiratory manifestations and lymphoedema. This article presents 52-year-old patient with membranous glomerulonephritis, hospitalized at the National Tuberculosis and Lung Diseases Research Institute in Warsaw, because of suspected allergic aspergillosis. Based on clinical and radiological evaluation the diagnosis of YNS was established.
View Article and Find Full Text PDFIntroduction: The diagnosis of Churg-Strauss syndrome (CSS) is difficult because pathological criteria are present in minority of patients and in advanced stages. Several centers elaborated criteria which allowed to suspect CSS in patients with asthma, hypereosinophilia and clinical manifestations consistent with systemic vasculitis with or without histologic evidence. The aim of the study is the presentation of the basis of CSS diagnosis in our material.
View Article and Find Full Text PDFThree patients with pleural sarcoidosis are reported. Pleural effusion in two patients and a massive pleural thickening that mimicked a tumour were observed. Histological examination of pleural biopsies revealed sarcoidosis.
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