Usual interstitial pneumonia uip refers to a morphologic entity defined by a combination of 1 patchy interstitial fibrosis with alternating areas of normal lung, 2 temporal heterogeneity of fibrosis characterized by scattered fibroblastic foci in the background of dense acellular collagen, and 3 architectural alteration due to chronic scarring or honeycomb change. Idiopathic interstitial pneumonias iips are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic patterns on lung biopsy. Atypical interstitial pneumonia definition of atypical. Symptoms of acute interstitial pneumonia including 4 medical symptoms and signs of acute interstitial pneumonia, alternative diagnoses, misdiagnosis, and correct diagnosis for acute interstitial pneumonia signs or acute interstitial pneumonia symptoms. Pneumonia is caused by a bacteria, virus or chemical. Usual interstitial pneumonia uip is the prototype of pulmonary fibrosis with socalled temporal heterogeneity. May 22, 2014 to illustrate the clinical and radiological features of idiopathic interstitial pneumonias iips, according to the american thoracic society atseuropean respiratory society ers classification updated in 20. The term acute interstitial pneumonia aip describes an idiopathic clinicopathological condition, characterized clinically by an interstitial lung disease causing rapid onset of respiratory. Acute interstitial pneumonitis is often categorized as both an interstitial lung disease and a form of acute respiratory distress syndrome ards but it is distinguished from the chronic forms of interstitial pneumonia such as idiopathic pulmonary fibrosis. Usual interstitial pneumonia an overview sciencedirect. It provides assessment of the potential etiology, extent, and distribution of disease, and is more likely to detect underlying or coexisting disease eg, occult mediastinal adenopathy, cancer, emphysema.
Acute interstitial pneumonitis aip, also known as hammanrich syndrome is a rapidly progressive noninfectious interstitial lung disease of unknown etiology. An integrated approach in the diagnosis of smokingrelated. Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy men and women, usually. On hrct images, usual interstitial pneumonia uip is characterized by the presence of reticular opacities, often associated with traction bronchiectasis. Management of the idiopathic interstitial pneumonias. Clinical management of acute interstitial pneumonia. Stanford medicine school of medicine departments surgical pathology criteria usual interstitial pneumonia navigation for this section. Interstitial pneumonia, desquamative definition of. They include primary fibrotic disorders, such as idiopathic pulmonary fibrosis, and primary inflammatory disorders, which may or may not be associated with lung fibrosis. Smoking relapse causing an acute exacerbation of desquamative. Nonspecific interstitial pneumonia cleveland clinic.
To illustrate the clinical and radiological features of idiopathic interstitial pneumonias iips, according to the american thoracic society atseuropean respiratory society ers classification updated in 20. Thus, idiopathic pulmonary fibrosis ipf and nonspecific interstitial pneumonia nsip, respiratory bronchiolitisinterstitial lung disease rbild. Temporal heterogeneity refers to the variegated appearance of the lung biopsy in uip, where areas of advanced fibrosis are seen adjacent to entirely normal lung, with interspersed areas of active fibroblastic proliferation known as fibroblastic foci. Acute interstitial pneumonia shows diffuse alveolar damage, which is almost completely identical to acute respiratory distress syndrome diffuse alveolar damage morphologically eur respir j 2000. In the united states, pneumonia is the sixth most common disease leading to death. Interstitial pneumonia with autoimmune features american. Pdf clinical management of acute interstitial pneumonia. Usual interstitial pneumonia radiology reference article. Acute interstitial pneumonia genetic and rare diseases. Because of these complexities, it is not easy to make the diagnosis of the. Aip is similar in presentation to the acute respiratory distress syndrome ards and probably. By and large, iip team is divided into seven distinct groupsidiopathic pulmonary fibrosis ipf, nonspecific interstitial pneumonia nsip. Druginduced interstitial lung disease diild occurs as a result of numerous agents, but the risk often only becomes. Aip is classified as an idiopathic interstitial pneumonia iip, and among the iips, it has the most acute onset and rapidly progressive course.
Cop, acute interstitial pneumonia aip, respiratory bronchiolitisassociated interstitial lung disease rbild, desquamative interstitial pneumonia dip, and lymphoid interstitial pneumonia lip. Highresolution computed tomography features of nonspecific interstitial pneumonia and usual interstitial pneumonia. Diagnostic approaches to diffuse interstitial lung diseases. Flaherty, andrei, king, et al diagnosing idiopathic interstitial pneumonia 1055 patients underwent a history, physical examination, complete pulmonary function testing, hrct, and slb. Uip is thus classified as a form of interstitial lung disease. It is synonymous with hammanrich syndrome, demonstrating no sex predominance. Usual interstitial pneumonia idiopathic pulmonary fibrosis nonspecific interstitial pneumonia nonspecific interstitial pneumonia respiratory bronchiolitis rb rb associated ild rbild desquamative interstitial pneumonia desquamative interstitial pneumonia diffuse. It is synonymous with hammanrich syndrome, demonstrating no sex predominance or correlation with smoking and. Acute interstitial pneumonia aip is a rare and fulminant form of diffuse lung injury originally described by hamman and rich in 1935.
Aip is defined histologically by organizing diffuse alveolar damage, a nonspecific pattern that occurs in other causes of lung injury unrelated to idiopathic interstitial pneumonia. Pathology of idiopathic interstitial pneumonias ncbi. The idiopathic interstitial pneumonias iips are a group of di. All interstitial lung diseases affect the interstitium, a part of your lungs. Interstitial pneumonia atlas of swine pathology pig333.
It may occur when an injury to the lungs triggers an abnormal healing. Acute interstitial pneumonia aip is a rare and serious condition that affects the lungs. This atlas of swine pathology features images of major diseases and aims to be an eminently visual and very useful tool for diagnosis. These air sacs, called the alveoli, are where the exchange of oxygen and carbon dioxide takes place between the lungs and the bloodstream. Interstitial pneumonia how is interstitial pneumonia abbreviated. Patients without an hrct scan or an slb were excluded. Jul 22, 2017 acute interstitial pneumonia aip is a rare and serious condition that affects the lungs. The effectiveness of cpa in interstitial pneumonia associated with collagen vascular disease is occasionally reported, but the effect on idiopathic interstitial pneumonia, especially in acute and. Idiopathic interstitial pneumonias radiology reference.
Lymphoid interstitial pneumonia can also occur in adults, often those with autoimmune disorders such as plasma cell disorders, sjogren syndrome, hashimoto thyroiditis, rheumatoid arthritis, and systemic lupus erythematosus lupus. Idiopathic interstitial pneumonia, american thoracic. Acute interstitial pneumonia aip is an idiopathic interstitial lung disease that is clinically characterized by sudden onset of dyspnea and rapid development of respiratory failure. Hammanrich syndrome, also known as acute interstitial pneumonia, is a rare and fulminant form of idiopathic interstitial lung disease. Another name for interstitial lung disease or close medical condition association. Classified into 8 histologic subtypes, all are characterized by varying degrees of inflammation and fibrosis and all cause dyspnea. The most common symptoms are a dry cough and shortness of breath. Acute interstitial pneumonia merck manuals professional edition. In 1935, hamman and rich first reported autopsy cases of initially healthy individuals who developed a rapidly progressive and fatal type of interstitial lung disease, which differed from other interstitial pneumonia clinically and pathologically trans am clin climatol assoc 1935. Signs of pneumonia breathing faster than normal or having trouble breathing pain in the chest when breathing or.
In normal lungs, this tissue is very thin to allow oxygen and carbon dioxide to cross between the surface of the alveoli air sacs and the capillaries that carry blood. Acute interstitial pneumonia pulmonary disorders msd. Acute interstitial pneumonitis radiology reference article. Martinez university of michigan health system, ann arbor, michigan diffuseparenchymallungdiseasesdpldsareagroupofdisorders that involve the space between the epithelial and endothelial basement membranes. Interstitial lung disease is recognized most commonly in middle to old age west highland white terriers pneumocystis cariniiminiature dachshunds less than 1 year of age at risk endogenous lipid pneumonia is a type of interstitial pneumonia characterized by the presence of macrophages. Interstitial lung disease ild, or diffuse parenchymal lung disease dpld, is a group of lung diseases affecting the interstitium the tissue and space around the alveoli air sacs of the lungs. To determine whether the effusion is transudate or exudate. Idiopathic pulmonary fibrosisusual interstitial pneumonia. Acute interstitial pneumonia aip is a rare and fulminant idiopathic pulmonary disorder that manifests similarly to acute respiratory distress syndrome ards. Age and sex distribution acute interstitial pneumonia is a rare disorder that typically affects individuals over the age of 40 years. Lymphoid interstitial pneumonia is an uncommon lung disease in which mature lymphocytes a type of white blood cell accumulate in the air sacs of the lungs.
These include bacteria, amoebae, viruses, fungi, and parasites. Acute interstitial pneumonia, also commonly referred to as hammanrich syndrome, is a fulminant lung disease of unknown etiology occurring in previously healthy patients. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Data collection a standard form was used to collect clinical information, including.
The idiopathic interstitial pneumonias iip is defined as a group of chronic, progressive diffuse parenchymal lung diseases with unclear cause, characterized by expansion of the interstitial compartment of inflammatory cells, and is potential to develop pulmonary fibrosis in many cases. Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy men and women, usually those 40 years. The european respiratory societyamerican thoracic society task force on undifferentiated forms of. Elliot tl, lynch da, newell jd jr, cool c, tuder r, markopoulou k, veve r, brown kk. Architectural distortion suggestive of lung fibrosis is also frequently recognized. Dec 18, 2012 acute interstitial pneumonia, which occurs over a wide range of ages, with an approximate mean age of 50, early characterized by a viral upper respiratory infection with constitutional symptoms, soon develops respiratory failure over a couple of days and within weeks. Interstitial lung disease is the name for a group of diseases that affect the lungs, for example, interstitial pneumonitis, black lung, farmers lung, mold, grasses, fumes, and autoimmune diseases. The signs and symptoms generally develop and progress rapidly.
Idiopathic interstitial pneumonias usual interstitial pneumonia versus nonspecific interstitial pneumonia fernando j. Desquamative interstitial pneumonia dip is a rare interstitial. Usual interstitial pneumonia idiopathic pulmonary fibrosis nonspecific interstitial pneumonia nonspecific interstitial pneumonia respiratory bronchiolitis rb rb associated ild rbild desquamative interstitial pneumonia desquamative interstitial pneumonia diffuse alveolar damage acute interstitial pneumonia. Interstitial pneumonia how is interstitial pneumonia. Differential diagnosis usual interstitial pneumonia. It is localized to a relatively small area of the chest. It should be considered as a cause of idiopathic acute. Aug 09, 2016 interstitial lung disease is the name for a group of diseases that affect the lungs, for example, interstitial pneumonitis, black lung, farmers lung, mold, grasses, fumes, and autoimmune diseases. Usual interstitial pneumonia navigation for this section. Idiopathic interstitial pneumonia, idiopathic pulmonary fibrosis, usual interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis, rbild, acute interstitial pneumonia, hammanrich. Acute interstitial pneumonia, which occurs over a wide range of ages, with an approximate mean age of 50, early characterized by a viral upper respiratory infection with constitutional symptoms, soon develops respiratory failure over a couple of days and within weeks. Usual interstitial pneumonia uip is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis ipf on imaging, uip usually presents with a lung volume loss and a craniocaudal gradient of peripheral septal thickening, bronchiectasis, and honeycombing.
Overview of idiopathic interstitial pneumonias pulmonary. Usual interstitial pneumonia uip is a form of lung disease characterized by progressive scarring of both lungs. Pneumonia definition pneumonia is an infection of the lung that can be caused by nearly any class of organism known to cause human infections. The idiopathic interstitial pneumonias iips are diffuse interstitial lung diseases of unknown cause. The idiopathic interstitial pneumonias are characterised by varying degrees of lung inflammation and fibrosis. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. Clinical and radiological features of idiopathic interstitial. The interstitium in the lung is the tissue that lies between the surface of the alveoli and the capillaries that surround the alveoli.
Treatment and prognosis depends upon the type of lung disease. This page includes the following topics and synonyms. Interstitial lung disease ild is a group of many lung conditions. They are characterized by cellular infiltration of the interstitial compartment of the lung with varying degrees of inflammation and fibrosis. Usual interstitial pneumonia an overview sciencedirect topics. In the early stages of the condition, affected people may experience upper respiratory andor virallike symptoms such as cough, shortness of breath, and fever. Lymphoid interstitial pneumonia merck manuals consumer version. Acute interstitial pneumonia pulmonary disorders merck. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Idiopathic interstitial pneumonia, idiopathic pulmonary fibrosis, usual interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis, rbild, acute interstitial pneumonia, hammanrich disease, nonspecific interstitial pneumonia. Clinically there is acute or chronic respiratory distress, gross abnormality of lung sounds, and a complete absence of toxemia and other systemic signs that are characteristic of most other.
Hrct, which distinguishes airspace from interstitial disease, is the most useful test and is always done. The scarring involves the supporting framework interstitium of the lung. It is considered the only acute process among the idiopathic interstitial pneumonias. Groundglass attenuation, if present, is less extensive than reticular abnormality. Pneumoniaassociated acute glomerulonephritis request pdf. Jan 02, 2020 usual interstitial pneumonia uip refers to a morphologic entity defined by a combination of 1 patchy interstitial fibrosis with alternating areas of normal lung, 2 temporal heterogeneity of fibrosis characterized by scattered fibroblastic foci in the background of dense acellular collagen, and 3 architectural alteration due to chronic. Efficacy and safety of a decision rule for using antibiotics in children.
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