HypertensionLymphangioleiomyomatosisLangerhansArterialFibrosisParenchymaNontuberculous mycobacterial pulmonary diseaseDiseaseSystemic sclerosisDisordersDiseasesInflammationLungsInvolve the pulmonaryInvolvementCapillaryRenalEndothelial cellsDiagnosisArteriesMesenchymalAngiomyolipomaTuberousPerivascularArteryEmbolismAbnormalVasculitisAlveoliPathologyComposed of blood vesselsPhenotypeInducePneumothoraxGenesHamartomasProliferationCollagenPersistentCapillaries
Hypertension21
- Signs of pulmonary arterial hypertension with right ventricular dysfunction, such as lower extremity edema or jugular venous distention, may occur late in the course of any ILD and are not helpful in the diagnosis of a specific ILD. (thoracickey.com)
- In systemic sclerosis (SSc) patients, the change in the trend of scleroderma-related deaths over the past 30 years demonstrates that lung involvement (both pulmonary hypertension and pulmonary fibrosis) is among the primary causes of mortality [ 6 ]. (ersjournals.com)
- In SSc, pulmonary disease can exist as a heterogeneous combination of pulmonary fibrosis and pulmonary arterial hypertension (PAH), but the pathogenic mechanisms that induce different pulmonary responses are poorly understood. (ersjournals.com)
- Proline and glucose metabolic reprogramming supports vascular endothelial and medial biomass in pulmonary arterial hypertension. (oldhamlab.org)
- Integrating hemodynamics identifies an extreme pulmonary hypertension phenotype. (oldhamlab.org)
- Precision medicine in pulmonary hypertension. (oldhamlab.org)
- Pulmonary vascular distensibility and early pulmonary vascular remodeling in pulmonary hypertension. (oldhamlab.org)
- Persistent pulmonary hypertension (PPHN) is an important cause of neonatal mortality amongst infants who are of term or post-term gestation. (annals.edu.sg)
- This study aims to better understand breathing problems and blood flow through the heart in premature newborns to better detect conditions such as pulmonary hypertension. (nih.gov)
- Pulmonary hypertension is a condition in which blood pressure in the arteries of the lungs (the pulmonary arteries) is abnormally high. (msdmanuals.com)
- Many disorders can cause pulmonary hypertension. (msdmanuals.com)
- If the pressure of the blood in the pulmonary arteries increases to a sufficiently high level, the condition is called pulmonary hypertension. (msdmanuals.com)
- In pulmonary hypertension, the right side of the heart must work harder to push the blood through the pulmonary arteries. (msdmanuals.com)
- Cor Pulmonale Cor pulmonale is enlargement and thickening of the ventricle on the right side of the heart resulting from an underlying lung disorder that causes pulmonary hypertension (high pressures in the. (msdmanuals.com)
- Pulmonary arterial hypertension can be caused by numerous different disorders. (msdmanuals.com)
- Women are affected by idiopathic pulmonary hypertension twice as often as men, and the average age at which the diagnosis is made is about 35 years. (msdmanuals.com)
- The actual mechanism by which these inherited genetic mutations cause pulmonary hypertension is not yet known. (msdmanuals.com)
- A number of drugs and toxins have been identified as risk factors for pulmonary arterial hypertension such as fenfluramine (and other related weight-loss drugs), amphetamines, protein kinase inhibitors (such as dasatinib ), cocaine , and selective serotonin reuptake inhibitors (SSRIs). (msdmanuals.com)
- Persistent Pulmonary Hypertension of the Newborn Persistent pulmonary hypertension of the newborn is a serious disorder in which the arteries to the lungs remain narrowed (constricted) after delivery, thus limiting the amount of blood flow. (msdmanuals.com)
- Less common manifestations include hypertensive renal crisis, pulmonary hypertension or interstitial lung disease, and cardiomyopathy. (logicalimages.com)
- Limited cutaneous systemic sclerosis - distal skin sclerosis, Raynaud phenomenon, frequent severe late-stage complications such as pulmonary hypertension and gastrointestinal involvement. (logicalimages.com)
Lymphangioleiomyomatosis7
- and rare forms of ILD such as lymphangioleiomyomatosis (LAM) or Langerhans cell histiocytosis (LCH) account for the remaining subgroups. (springer.com)
- However, some diseases, such as sarcoidosis and lymphangioleiomyomatosis (LAM), may have only decreased breath sounds without adventitious sounds despite a markedly abnormal chest radiograph. (thoracickey.com)
- This contains not only ailments with well-defined clinical pathologic features, such as sarcoidosis, pneumoconiosis, lymphangioleiomyomatosis (LAM), or pulmonary Langerhans cell histiocytosis (PLCH), but also a cluster of common disorders as "idiopathic interstitial pneumonia" (IIP). (lungswiki.com)
- Pulmonary lesions are lymphangioleiomyomatosis (LAM) and pulmonary cysts. (medscape.com)
- Finally, a number of very rare forms of DPLDs exist, including pulmonary Langerhans cell histiocytosis (PLCH) (see Eosinophilic Granuloma (Histiocytosis X) ), tuberous sclerosis, lymphangioleiomyomatosis (LAM) (see Lymphangioleiomyomatosis ), and Hermansky-Pudlak syndrome. (medscape.com)
- The various etiologies identified were lymphangioleiomyomatosis (LAM), lymphocytic interstitial pneumonia, Birt-Hogg-Dube syndrome, hypersensitivity pneumonitis, Pneumocystis jirovecii pneumonia, and cystic metastasis, and in one patient, no definite cause was found despite detailed evaluation. (ijcdas.com)
- Lymphangioleiomyomatosis (LAM) is a rare disease. (afpm.org.my)
Langerhans1
- PLCH, pulmonary Langerhans cell histiocytosis. (thoracickey.com)
Arterial1
- Arterial hypoxemia in disorders of pulmonary parenchyma is primarily caused by ventilation-perfusion mismatching, with further contribution from an intrapulmonary shunt. (medscape.com)
Fibrosis15
- Idiopathic pulmonary fibrosis (31%) and connective-tissue disease related ILD (21.7%) were the two most common subtypes. (springer.com)
- Firstly, there are the Idiopathic Interstitial Pneumonias (IIPs) including Idiopathic Pulmonary Fibrosis (IPF), the most common IIP, along with idiopathic non-specific idiopathic pneumonia (iNSIP), acute interstitial pneumonia (AIP) and respiratory bronchiolitis-associated ILD (RB-ILD), to name a few. (springer.com)
- When responding to any injury-whether from a specific exposure (e.g., asbestos, nitrofurantoin, or moldy hay), an autoimmune-mediated inflammation from a systemic connective tissue disease (e.g., rheumatoid arthritis), or unknown injury (e.g., idiopathic pulmonary fibrosis [IPF])-the lung must respond to the damage and repair itself. (thoracickey.com)
- When the alveoli are damaged, thick-walled cysts are abandoned, causing the lungs to simulate a honeycomb and eventually leading to a treatment of pulmonary fibrosis. (lungswiki.com)
- cIdiopathic pulmonary fibrosis. (medscape.com)
- Pulmonary fibrosis share the pathogenesis process of interstitial lung disease which involve the pulmonary parenchyma . (wikidoc.org)
- Idiopathic pulmonary fibrosis (IPF) represents probably the most aggressive form of ILD and systemic sclerosis is a multiorgan fibrotic disease frequently associated with ILD. (ersjournals.com)
- Some forms of fibrosis, such as acute lung injury or cryptogenic organising pneumonia, are at least partially reversible, whereas others, in particular idiopathic pulmonary fibrosis (IPF), are progressive and fatal. (ersjournals.com)
- This schematic view of the morpho-functional unit of the lung (alveolus) depicts the main differences in cellular composition in idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) compared with normal physiological cellular components. (ersjournals.com)
- Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases (ILDs) or diffuse parenchymal lung diseases. (atsjournals.org)
- Idiopathic pulmonary fibrosis (or cryptogenic fibrosing alveolitis) (IPF or CFA) is one of several idiopathic interstitial pneumonias. (atsjournals.org)
- The purpose of this consensus statement is to provide assistance to clinicians in the diagnosis and management of idiopathic pulmonary fibrosis (IPF). (atsjournals.org)
- DPLD may be idiopathic, a classic illustration of which is idiopathic interstitial fibrosis (IPF), which is discussed in another article (see Pulmonary Fibrosis, Idiopathic ). (medscape.com)
- This article presents a broad overview, with an emphasis on those etiologies that result in pulmonary fibrosis not discussed elsewhere in this series. (medscape.com)
- It is thought to begin with acute injury to the pulmonary parenchyma, leading to chronic interstitial inflammation, then to fibroblast activation and proliferation, and finally progressing to the common endpoint of pulmonary fibrosis and tissue destruction. (medscape.com)
Parenchyma4
- Figure 24-2 illustrates the components of the normal pulmonary parenchyma. (thoracickey.com)
- A, Diagram of the pulmonary parenchyma shows the respiratory bronchiole, alveolar duct, and alveolar sacs. (thoracickey.com)
- Interstitial lung disease is a group of disorders that involve pulmonary parenchyma . (wikidoc.org)
- These arteries (except the thyroid artery) form a peribronchial plexus that follows the bronchial tree deep into the lung parenchyma to supply blood also to the visceral pleura and the walls of the pulmonary arteries and veins (vasa vasorum). (medscape.com)
Nontuberculous mycobacterial pulmonary disease3
- The incidence and prevalence of nontuberculous mycobacterial pulmonary disease (NTM-PD) have been increasing worldwide. (ersjournals.com)
- Higher body mass index was protective against development of nontuberculous mycobacterial pulmonary disease (NTM-PD) among Korean people. (ersjournals.com)
- Typically, NTM infection causes progressive inflammatory damage to the lung, resulting in nontuberculous mycobacterial pulmonary disease (NTM-PD). (ersjournals.com)
Disease16
- The interstitium (the pace tissue around the air sacs) of the lungs is affected by interstitial lung disease. (lungswiki.com)
- Some of the other names for this condition are cryptogenic fibrosing alveolitis (CFA), diffuse interstitial lung disease, idiopathic pulmonary pneumonitis (IPP), and alveolitis. (lungswiki.com)
- Idiopathic fibrotic illnesses, drug-induced lung disease, connective tissue disorders, and different other primary lung diseases are examples of such diseases. (lungswiki.com)
- Unlike obstructive lung diseases, such as asthma and chronic obstructive pulmonary disease (COPD), which show a normal or increased total lung capacity (TLC), restrictive disease are associated with a decreased TLC. (medscape.com)
- The diseases cause inflammation or scarring of the lung tissue (interstitial lung disease) or result in filling of the air spaces with exudate and debris (pneumonitis). (medscape.com)
- They include idiopathic fibrotic diseases, connective-tissue diseases, drug-induced lung disease, environmental exposures (inorganic and organic dusts), and primary diseases of the lungs (including sarcoidosis). (medscape.com)
- Frequently, however, ILDs can also be associated with a specific environmental exposure or an underlying connective tissue disease. (ersjournals.com)
- Scleroderma is an aspect of systemic sclerosis , a systemic connective tissue disease that also involves subcutaneous tissue, muscles, and internal organs. (medscape.com)
- The term systemic sclerosis is used to describe a systemic autoimmune disease of unknown origin characterized by excessive deposition of collagen and other connective tissue macromolecules in skin and multiple internal organs, prominent and often severe fibroproliferative alterations in the microvasculature, and numerous humoral and cellular immunologic abnormalities. (medscape.com)
- Systemic vascular distensibility relates to exercise capacity in connective tissue disease. (oldhamlab.org)
- Of patients referred to a pulmonary disease specialist, an estimated 10-15% have a DPLD. (medscape.com)
- In addition, poor nutritional status is correlated with the progression of NTM-PD [ 13 ] and higher all-cause mortality among patients with Mycobacterium avium complex pulmonary disease [ 14 ]. (ersjournals.com)
- Scleroderma, or progressive systemic sclerosis, is an autoimmune connective tissue disease that involves sclerotic changes of the skin and may involve internal organs. (logicalimages.com)
- Pulmonary disease is the leading cause of mortality. (logicalimages.com)
- The disease is characterised by an abnormal proliferation of smooth muscle-like cells that grow over a course of time to obstruct airways, lymphatic and blood vessels. (afpm.org.my)
- Corrigendum to "Chronic obstructive pulmonary disease is associated with a higher risk of functional gastrointestinal disorders" [Respir. (tmu.edu.tw)
Systemic sclerosis2
- New insights into the complex cellular contributions and interactions will be provided, comparing the role of cell subsets in the pathogenesis of IPF and systemic sclerosis. (ersjournals.com)
- Systemic Sclerosis Systemic sclerosis is a rare, chronic autoimmune connective tissue disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities. (msdmanuals.com)
Disorders4
- Macrophages, white blood cells, and protein-rich fluid collect in the interstitial spaces in the early stages of various interstitial lung disorders, causing inflammation. (lungswiki.com)
- Although the aetiology of these disorders remains unknown, in this review we analyse the pathogenic mechanisms by cell of interest (fibroblast, fibrocyte, myofibroblast, endothelial and alveolar epithelial cells and immune competent cells). (ersjournals.com)
- Adult Mesenchymal Stem Cells Engineering for Connective Tissue Disorders. (health-abstracts.com)
- Six genes carrying DNVs were associated with human developmental disorders involving epithelial, connective or bone morphologies ( PXDN, RTEL1, ANKRD11, MAP2K1, CYLD, ACAN) and four linked with cholangio- and hepatocellular carcinomas (PIK3CA, TLN1 CYLD, MAP2K1) . (biomedcentral.com)
Diseases2
- Whether associated with these diseases or sporadic, angiomyolipomas are caused by mutations in either the TSC1 or TSC2 genes, which govern cell growth and proliferation. (wikipedia.org)
- Panel members are experts in adult pulmonary diseases. (atsjournals.org)
Inflammation2
- Intrinsic lung illnesses are those that induce scarring of the lung tissue or inflammation of the lung tissue, as well as the accumulation of debris and exudate in the alveoli. (lungswiki.com)
- Therefore, efficient detection and clearance of apoptotic cells (ACs), a process termed efferocytosis, is critical for control of tissue homeostasis and the resolution of inflammation. (keystonesymposia.org)
Lungs3
- In all the above-mentioned conditions, the deep, inner tissues of the lungs become inflamed. (lungswiki.com)
- Blood travels from the right side of the heart through the pulmonary arteries into the small blood vessels of the lungs (the capillaries) where carbon dioxide is removed from the blood and oxygen is added. (msdmanuals.com)
- Normally, the pressure in the pulmonary arteries is low, allowing the right side of the heart to be less muscular than the left side (because relatively little muscle and effort are needed to push the blood through the lungs via the pulmonary arteries). (msdmanuals.com)
Involve the pulmonary1
- As the name ILD implies, the histologic abnormalities that characterize ILD involve the pulmonary interstitium to a greater extent than the alveolar spaces or airways, although exceptions exist. (thoracickey.com)
Involvement1
- Pulmonary involvement is frequent. (lookformedical.com)
Capillary1
- B, The constituents of the interstitial space, including type I and type II alveolar epithelial cells, a capillary with vascular endothelial cells and erythrocytes in transit, resident macrophages, interstitial fibroblasts, and matrix substance. (thoracickey.com)
Renal2
- Distinguishing a fat-poor angiomyolipoma from a renal cell carcinoma (RCC) can be difficult. (wikipedia.org)
- Common features include cortical tubers, subependymal nodules (SENs), subependymal giant cell astrocytomas (SEGAs), facial angiofibromas, hypomelanotic spots known as Fitzpatrick patches (ash-leaf spots), cardiac rhabdomyomas, and renal angiomyolipomas. (medscape.com)
Endothelial cells1
- 2013). "Comparing the differential effects of LPA on the barrier function of human pulmonary endothelial cells" . (wikidoc.org)
Diagnosis1
- Chest x-rays, electrocardiography (ECG), and echocardiography give clues to the diagnosis, but measurement of blood pressure in the right ventricle and the pulmonary artery is needed for confirmation. (msdmanuals.com)
Arteries1
- Cutaneous polyarteritis nodosa (CPN) is an uncommon form of vasculitis of the small-and medium-sized arteries in the reticular dermis and subcutaneous tissue. (annals.edu.sg)
Mesenchymal3
- PEComas are themselves a kind of mesenchymal tumour which involves cells that form the connective tissue, cardiovascular, and lymphatic systems. (wikipedia.org)
- A family of mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. (curehunter.com)
- Haematopoietic and mesenchymal stem-cell. (health-abstracts.com)
Angiomyolipoma2
- An angiomyolipoma is composed of varying proportions of vascular cells, immature smooth muscle cells, and fat cells. (wikipedia.org)
- Since all three components of an angiomyolipoma (vascular cells, immature smooth muscle cells, and fat cells) contain a "second-hit" mutation, they are believed to have derived from a common progenitor cell that developed the common second-hit mutation. (wikipedia.org)
Tuberous1
- Angiomyolipomas, found in 70-80% of patients with tuberous sclerosis complex, are composed of blood vessels, smooth muscle, adipose tissue, and connective tissue. (medscape.com)
Perivascular2
- Angiomyolipomas are tumours consisting of perivascular epithelioid cells (cells which are found surrounding blood vessels and which resemble epithelial cells). (wikipedia.org)
- A tumour of this kind is known as a PEComa, from the initials of perivascular epithelioid cell. (wikipedia.org)
Artery1
- Pulmonary embolism is the obstruction of the pulmonary artery or its branches, commonly by thrombus or fat. (afpm.org.my)
Embolism1
- We report an unusual case of double pathology - both pulmonary thromboembolism and fat embolism syndrome in a patient with bilateral femur and bilateral tibia fractures. (afpm.org.my)
Abnormal4
- If the initiating injury or abnormal repair from injury is not halted, progressive tissue damage leading to worsening physiologic impairment and death can occur. (thoracickey.com)
- The hamartomas in the brain called cortical tubers are composed of abnormal glial and neural cells, and the size, number, and location vary among patients. (medscape.com)
- These lesions are composed of blood vessels, adipose tissue, and smooth muscle in abnormal arrangements. (medscape.com)
- Abnormal increase of EOSINOPHILS in the blood, tissues or organs. (lookformedical.com)
Vasculitis1
- Necrotizing vasculitis mediated by cytotoxic T cells, leading to ischaemic changes, appears to be a major cause of Churg-Strauss syndrome-associated neuropathy. (lookformedical.com)
Alveoli1
- If the exposure or injury persists or if the injury repair process is imperfect, the lung may be permanently damaged with increased interstitial tissue replacing the normal capillaries, alveoli, and healthy interstitium. (thoracickey.com)
Pathology1
- World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone, 2002). (curehunter.com)
Composed of blood vessels1
- They are composed of blood vessels, smooth muscle cells, and fat cells. (wikipedia.org)
Phenotype1
- CAFs maintain their phenotype for numerous passages during culture in vitro without exposure to cancer cells, while NFs cannot be infinitely proliferous like cancer cells ( 10 ). (spandidos-publications.com)
Induce1
- It is widely recognized that the accumulation of various harmful genetic alterations in normal cells may induce malignant cancer cells ( 1 ). (spandidos-publications.com)
Pneumothorax1
- We report a case of LAM in a young woman who presented with a spontaneous pneumothorax. (afpm.org.my)
Genes4
- This study aims to learn more about the impact of changes in connective tissue genes on an individual's overall health. (nih.gov)
- En ese sentido, existe un creciente enfoque en la identificación de genes, polimorfismos genéticos asociados y loci de susceptibilidad múltiples. (scielo.br)
- La presente revisión destaca los genes y polimorfismos genéticos actualmente estudiados, identificados como influyentes en la génesis de la EI, como los genes MMP-3, IL-6, colágeno tipo 1 y receptores de vitamina D y de estrógeno. (scielo.br)
- Of the 31 genes, the 21 upregulated genes were primarily associated with cell paracrine and intracellular signaling, transcription regulation and cell adhesion and migration, and their transcriptional products included transforming growth factor-β2 (TGF-β2), insulin-like growth factor-binding protein 2 and transcriptional factor AP-2α/γ ( 11 ). (spandidos-publications.com)
Hamartomas1
- Older literature may classify them as hamartomas (benign tumours consisting of cells in their correct location, but forming a disorganised mass) or choristoma (benign tumours consisting of normal cells in the wrong location). (wikipedia.org)
Proliferation1
- CAFs provide cancer cells with nutrition and promote the proliferation, invasion and metastasis of cells ( 6 - 9 ). (spandidos-publications.com)
Collagen1
- Normally, collagen is degraded and produced regularly to preserve the normal lung tissue. (wikidoc.org)
Persistent1
- It may achieve sufficient transcriptional repression to maintain persistent infection and long-term latency of HTLV-1 in the host cells. (bvsalud.org)
Capillaries1
- Figure 24-2 shows, in the normal state, this space allows close apposition of gas and capillaries with minimal connective tissue matrix, fibroblasts, and inflammatory cells such as macrophages. (thoracickey.com)