Browse Pulmonary Fibrosis
Filter Results
- By Article Category
- All Categories
- Clinical Cases (88)
- Research (41)
- Commentary (11)
- Review (8)
- Other (7)
- By Date
- Past 10 years
- Past 20 years
- Past 50 years
- Past 100 years
- Complete archive (1812-present)
- Specific date range
Sort By:
- Newest
- Oldest
- Most Viewed
- Most Cited
Original Article
Prednisone, Azathioprine, and N-Acetylcysteine for Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a chronic, progressive lung disease of unknown cause characterized by the histopathological pattern of usual interstitial pneumonia. The median survival of patients with idiopathic pulmonary fibrosis after diagnosis is 2 to 5 years. The use of glucocorticoids or…
Correspondence
Pulmonary Fibrosis, Bone Marrow Failure, and Telomerase Mutation
To the Editor: Telomeres protect the ends of chromosomes from erosion; telomerase ensures their integrity. We report a case of familial idiopathic pulmonary fibrosis and bone marrow failure associated with a mutation in telomerase reverse transcriptase (TERT). The patient was a 56-year-old lifelong…
- Free Full Text
Original Article
Efficacy of a Tyrosine Kinase Inhibitor in Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a debilitating disease characterized by destruction of the gas-exchanging regions of the lung. Its pathogenesis is thought to involve aberrant wound healing mediated by multiple signaling pathways, resulting in progressive lung injury and scarring. Symptoms,…
- Free Full Text
Editorial
Resolving the Scar of Pulmonary Fibrosis
Fibrosis, or scarring, of the lung is a common consequence of certain types of pneumonias, and in such cases it is a localized and self-limited process. However, in conditions such as the complex group of disorders termed the idiopathic interstitial pneumonias, pulmonary fibrosis may be a…
- Free Full Text
Correspondence
A Variant in the Promoter of MUC5B and Idiopathic Pulmonary Fibrosis
To the Editor: Idiopathic pulmonary fibrosis is a complex genetic disease; mutations in surfactant protein C, telomerase, and surfactant protein A2 have been identified in familial cases of pulmonary fibrosis. We confirm a recent association with rs35705950 in the putative promoter of MUC5B and…
- Free Full Text
Editorial
Idiopathic Pulmonary Fibrosis — A Sticky Business
Pulmonary fibrosis comprises a spectrum of disease phenotypes, including familial and idiopathic forms. Current research suggests that pulmonary fibrosis is a "two-hit" disease: a genetic predisposition to abnormal alveolar epithelial-cell regulation of the cell cycle and apoptosis combined with…
Original Article
A Common MUC5B Promoter Polymorphism and Pulmonary Fibrosis
The evidence that there is a genetic basis for idiopathic interstitial pneumonia is substantial, with familial aggregation confirmed through studies in twins, siblings raised apart, and multigenerational families. Interstitial lung disease has been associated with several pleiotropic genetic…
- Free Full Text
Editorial
Targeting the Basic Defect in Cystic Fibrosis
Therapies for cystic fibrosis have been limited to alleviating clinical manifestations, and although the duration and quality of patients' lives have improved, cystic fibrosis continues to inflict major burdens and shorten lives. In this issue of the Journal, Accurso and colleagues have taken a…
Original Article
Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation
Cystic fibrosis is a progressive lung disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, an epithelial ion channel involved in salt and fluid transport in multiple organs, including the lung. Current treatments for cystic…
- Free Full Text
- CME
Original Article
A Controlled Trial of Sildenafil in Advanced Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a chronic, progressive lung disease of unknown cause that is characterized by the histopathologic pattern of usual interstitial pneumonia. Progression to end-stage respiratory insufficiency and death within 5 years after the onset of symptoms is characteristic. To…
- Free Full Text
Case Records of the Massachusetts General Hospital
Case 24-2010 — A 56-Year-Old Woman with a History of Hodgkin's Lymphoma and Sudden Onset of Dyspnea and Shock
Presentation of Case. Dr. Karen M. Winkfield (Radiation Oncology): A 56-year-old woman with a history of childhood Hodgkin's lymphoma was admitted to the emergency department of this hospital because of the sudden onset of dyspnea and hypotension. The patient had been in her usual state of health…
- CME
A 56-year-old woman was admitted because of the sudden onset of dyspnea and hypotension. At 16 years of age, she had received combination chemotherapy and extended-field radiation therapy for Hodgkin's lymphoma. On admission, an electrocardiogram showed an acute myocardial infarction. A stent was placed, but hypotension and acidosis persisted, and the patient died.
Case Records of the Massachusetts General Hospital
Case 12-2010 — An 89-Year-Old Man with Progressive Dyspnea
Presentation of Case. An 89-year-old man was admitted to the hospital because of progressive dyspnea. The patient had been in his usual state of health, with diabetes mellitus, coronary artery disease, and complete heart block, until 6 months before admission, when shortness of breath developed.…
- Video
An 89-year-old man was admitted to the hospital because of progressive dyspnea for 6 months, worsening over the past 3 days. He had a history of exposure to asbestos and had smoked cigarettes for many years. Imaging studies revealed a pleural plaque and pulmonary interstitial fibrosis with superimposed ground-glass opacities. Hypoxemia and intermittent hypotension occurred. Despite oxygen supplementation, mechanical ventilation, and pressor administration, a cardiac arrest occurred, and the patient died on the eighth hospital day. An autopsy was performed.
Review Article
Mechanisms of Disease: Telomere Diseases
Elizabeth Blackburn, Jack Szostak, and Carol Greider were recently awarded the Nobel Prize in Physiology or Medicine for their elucidation of the structure and maintenance of telomeres (the tips of chromosomes). These investigators discovered that telomeres are DNA sequences with a structure that…
The Nobel Prize in Physiology or Medicine 2009 was recently awarded to three scientists for their work on telomeres, which are repetitive DNA structures at the tips of chromosomes, and the enzyme telomerase. Telomeres protect the genome from loss of genetic material. This review recounts the medical implications of telomeres and focuses on a group of diseases in which the loss of telomeres appears to be causal.
Case Records of the Massachusetts General Hospital
Case 32-2009 — A 27-Year-Old Man with Progressive Dyspnea
Presentation of Case. A 27-year-old man was seen in the outpatient pulmonary division of this hospital because of progressive dyspnea and decreased exercise tolerance. Approximately 2 years earlier, the patient noted a slight decrease in his exercise tolerance. He was able to exercise regularly and…
A 27-year-old man was seen at this hospital because of progressive dyspnea and decreased exercise tolerance during the past 2 years. Imaging studies showed diffuse pulmonary fibrosis. The patient had lighter skin than many in his family, although he reported that several family members had light skin and respiratory problems. He had a history of easy bruising. On examination, his skin was pale, and there was clubbing of the fingers and toes, normal breath sounds, pigmented irises, and horizontal nystagmus. A diagnostic procedure was performed.
Case Records of the Massachusetts General Hospital
Case 6-2009 — A 37-Year-Old Woman with Vertigo, Facial Weakness, and a Generalized Seizure
Presentation of Case. Dr. Sang-Woo (Steve) Han (Neurology): A 37-year-old woman was seen in the emergency department of this hospital because of a seizure. Four months earlier, episodes of vertigo began to occur several times a week that lasted up to several hours and were associated with nausea…
A 37-year-old woman had a 4-month history of episodes of vertigo, followed by tinnitus, decreased hearing in her right ear, and right facial weakness; on the day of admission, she had a generalized seizure. On admission, she was confused and combative and had garbled speech, a right facial droop, and generalized hyperreflexia. Magnetic resonance imaging of the brain and spinal cord revealed multifocal linear and nodular enhancement of the leptomeninges. A diagnostic procedure was performed.
Case Records of the Massachusetts General Hospital
Case 6-2008 — A 46-Year-Old Woman with Renal Failure and Stiffness of the Joints and Skin
Presentation of Case. Dr. Nancy Cibotti-Granof (Medicine): A 46-year-old woman with end-stage renal disease was seen by a rheumatology consultant because of stiffness of her joints and skin. The patient had been well except for mild asthma until 7 years earlier, when group A streptococcal pneumonia…
A 46-year-old woman was seen by a rheumatology consultant because of stiffness of the joints and skin. She had been well until 7 years earlier, when an episode of group A streptococcal pneumonia complicated by septic shock left her with chronic kidney disease and a painful peripheral neuropathy. Three years later, gradually progressive stiffness of the skin of her hands and feet developed, with pain and stiffness of the joints; renal failure worsened, and respiratory distress and congestive heart failure developed. A diagnostic procedure was performed.
Clinical Implications of Basic Research
Idiopathic Pulmonary Fibrosis — New Insights
"Death occurred about three months and a half after the onset of the acute disease and the lung was two thirds of the normal size, grayish in color, and hard as cartilage. Microscopically these areas showed advanced fibrotic changes and great thickening of the alveolar walls." Thus did Sir William…
Original Article
Telomerase Mutations in Families with Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis has a predictable, progressive clinical course that ultimately leads to respiratory failure. Irreversible fibrosis is the hallmark of the disease, which has a characteristic radiographic appearance most often associated with the pathological lesion of usual…
- Free Full Text
Mutations affecting both components of the telomerase enzyme, hTERT and hTR, are associated with familial idiopathic pulmonary fibrosis, and carriers of such mutations have shorter telomeres than do noncarrier family members. This finding suggests that the disease may be triggered by a loss of alveolar cells, the progenitors of which may be limited by short telomeres.
Original Article
High-Dose Acetylcysteine in Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a chronic progressive interstitial pneumonia with a poor prognosis.– It has been proposed that a pathogenetic mechanism of idiopathic pulmonary fibrosis is repeated lung injury, with aberrant progressive fibrotic reaction.– If this is the case, it may explain…
- Free Full Text
Idiopathic pulmonary fibrosis is a progressive disorder characterized by loss of lung function; there is no effective treatment. In this multinational, randomized, placebo-controlled trial with all patients receiving azathioprine and prednisone, acetylcysteine was shown to slow the rate of deterioration of lung function. There was no effect on survival.







