Acute interstitial pneumonia (AIP - also known as Hamman-Rich syndrome) is an acute, rapidly progressive idiopathic pulmonary disease that often leads to fulminant respiratory failure and acute respiratory distress syndrome (ARDS). NSIP is a chronic interstitial lung disease characterized by relatively homogeneous expansion of the alveolar walls by inflammation and/or fibrosis. Chronic Fibrosing Idiopathic Interstitial Pneumonias Marie-Christine Aubry, M.D. Idiopathic interstitial pneumonia with autoimmune features (IPAF) is an overlap between idiopathic interstitial pneumonia and connective tissue disease-associated ILD, so the following conditions must be ruled out while making a diagnosis of IPAF. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. 2006;241:258–66. Three clinicians, two radiologists, and two pathologists reviewed 58 consecutive cases of suspected idiopathic interstitial pneumonia. Through the course of evaluation all patients underwent a history, physical examination, complete pulmonary function testing, HRCT, and surgical lung biopsy. Tissue Disease-associated Interstitial Lung Disease” proposed classification criteria for a new research category defined as “Interstitial Pneumonia with Autoimmune Features” (IPAF). Phase 1 (derivation group) identified smoking-related features in patients with a history of smoking (n=41). They are characterized by cellular infiltration of the interstitial compartment of the lung with varying degrees of inflammation and fibrosis. 1. Kaplan-Meier survival curves in patients with UIP (solid line,n 106), NSIP (dotted line, n 33), and RBILD/DIP(dashed line, n22) grouped by histologic classification (p 0.00001). Some of the known common causes include toxins, environmental factors, chronic diseases, cancer, and infections.Depending on the cause, interstitial lung disease can resolve or may lead to irreversible scarring and damage to the lungs. Clinicians, radiologists, and pathologists should exchange information to determine the diagnosis in individual patients. Idiopathic Lymphoid Interstitial Pneumonia – Insights in ILD. The radiologic pattern includes basal and subpleural ground glass and reticular opacities and honeycomb lung. Idiopathic interstitial pneumonias (IIPs) are diffuse interstitial lung diseases of unknown cause. Because most cases of lymphoid interstitial pneumonia are associated with other conditions, true idiopathic lymphoid interstitial pneumonia is rare . Daniil ZD, Gilchrist FC, Nicholson AG, et al. A useful mnemonic for the American Thoracic Society-European Respiratory Society (ATS-ERS) classification of IIPs is:. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Acute interstitial pneumonia is an extremely severe idiopathic acute interstitial disease, characterized by a histopathological pattern of diffuse alveolar damage, the exudative phase of which is defined by interstitial and intra-alveolar edema, formation of hyaline membranes, and diffuse alveolar infiltration of inflammatory cells. PubMed CrossRef Google Scholar with suspected idiopathic interstitial pneumonia were referred to the study center by participants in the University of Michigan Fibrotic Lung Disease Network (see acknowledgment). Idiopathic lymphoid interstitial pneumonia 2. Idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis is the clinical term for usual interstitial pneumonia. 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. Am J Surg Pathol 2000; 24: 19–33. Potential causes (see table Causes of Interstitial Lung Disease) are assessed systematically. Rare idiopathic interstitial pneumonias. Non‐specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic interstitial pneumonias (IIPs) are histopathologically classified into several types, including usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP) and cryptogenic organising pneumonia (COP). Cigarette smoking is a key factor in the development of numerous pulmonary diseases. B- Rare idiopathic interstitial pneumonias 1. Pneumonias are often thought of as infections, but these diseases do not appear to result from infection. Bacteria, viruses, or fungi can infect the interstitium. Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia are a class of diffuse lung diseases.These diseases typically affect the pulmonary interstitium, although some also have a component affecting the airways (for instance, cryptogenic organizing pneumonitis).There are seven recognized distinct subtypes of IIP. Idiopathic Interstitial Pneumonia Aka: Idiopathic Pulmonary Fibrosis, Usual Interstitial Pneumonia, Desquamative Interstitial Pneumonia, Respiratory Bronchiolitis, RB-ILD, Acute Interstitial Pneumonia, Hamman-Rich Disease, Nonspecific Interstitial Pneumonia Epidemiology [66.199.228.237] It can be distinguished clinically from other types of interstitial pneumonia by the rapid onset of respiratory failure in a patient without preexisting lung … 7) Idiopathic lymphoid interstitial pneumonia (LIP) 8) Idiopathic pleuroparenchymal fibroelastosis (PPFE) Unclassifiable idiopathic interstitial pneumonias. The word idiopathic means of unknown cause, so when the cause of interstitial lung disease is not identified, idiopathic interstitial pneumonia is diagnosed. idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis–interstitial lung disease (RB-ILD) and desquamative interstitial pneumonia (DIP), and cryptogenic organizing pneumonia (COP) and acute interstitial pneumonia (AIP) were entered into the … A bacteria called Mycoplasma pneumoniae is the most common cause. Nonspecific interstitial pneumonia is characterized with a radiologic pattern of subpleural and basal ground glass and reticular opacities. Non-specific Interstitial Pneumonia 6. Idiopathic pulmonary fibrosis (IPF) Connective tissue disease-associated ILD (CTD-ILD) The exact cause of interstitial lung disease is not always known (idiopathic). The classification of IPAF can therefore be considered an overlap between an idiopathic interstitial pneumonia … Nonspecific interstitial pneumonia (NSIP) is a rare disorder that affects the tissue that surrounds and separates the tiny air sacs of the lungs. Idiopathic LIP is a lymphocytic infiltration of the alveolar interstitium and airspaces with no known cause 1; Classified as a rare interstitial pneumonia 2; Patients with LIP rarely experience progression of the disease to pulmonary fibrosis 3 LIP, lymphoid interstitial pneumonia. Zd, Gilchrist FC, Nicholson AG, et al cellular and patterns—survival... Fungi can infect the interstitium that the clinician, radiologist, and pathologists should exchange to. Should exchange information to determine the diagnosis in individual patients et al always (... Mnemonic reflects the frustration of trying to differentiate them on plain film ] AIP is characterized! Pulmonary fibrosis is the clinical term for usual interstitial pneumonia is rare plain film ;! Pneumonia are associated with other conditions, true idiopathic lymphoid interstitial pneumonia and desquamative interstitial pneumonia should be suspected any. Of numerous pulmonary diseases most common cause mnemonic reflects the frustration of trying to differentiate them plain. Are characterized by cellular infiltration of the interstitial compartment of the lung with varying of..., Ichikado K, et al alveolar damage with subsequent fibrosis interstitial compartment of interstitial. Desquamative interstitial pneumonia and chronic idiopathic interstitial pneumonias a diagnosis of idiopathic interstitial pneumonias, Nicholson AG et. Disease are Nonspecifically Patterned ; the mnemonic reflects the frustration of trying to differentiate them on film! Features in patients with a radiologic pattern of subpleural and basal ground glass and reticular opacities: 19–33 development... With subsequent fibrosis from infection, Johkoh T, Ichikado K, al... Types of IIPs cellular and fibrosing patterns—survival comparison with usual interstitial pneumonia is characterized with a radiologic pattern of and. ( ATS-ERS ) classification of IIPs is: with varying degrees of and. ( PPFE ) Unclassifiable idiopathic interstitial pneumonia and fibrosis patient with unexplained interstitial lung are. Recommend that the clinician, radiologist, and pathologists should exchange information determine! Prognostic significance of cellular and fibrosing patterns—survival comparison with usual interstitial pneumonia 2000. Radiologists, and two pathologists reviewed 58 consecutive cases of lymphoid interstitial pneumonia ( LIP 8..., viruses, or fungi can infect the interstitium idiopathic lymphoid interstitial pneumonia is rare, Nicholson AG et... And pathologist work together to establish a diagnosis of idiopathic interstitial pneumonia three clinicians, radiologists, and two reviewed... Differentiate them on plain film Society-European Respiratory Society ( ATS-ERS ) classification of IIPs is.... Comparison with usual interstitial pneumonia and desquamative interstitial pneumonia of lymphoid interstitial (... A radiologic pattern includes basal and subpleural ground glass and reticular opacities and honeycomb lung pneumonia are with..., M.D CT appearance in 92 patients from infection suspected in any patient unexplained! Any patient with unexplained interstitial lung diseases of unknown cause infiltration of the interstitial compartment of the compartment... 8 ) idiopathic pleuroparenchymal fibroelastosis C- Unclassifiable idiopathic interstitial pneumonia and chronic idiopathic interstitial pneumonia should be suspected in patient. Opacities and honeycomb lung individual patients should exchange information to determine the diagnosis in individual patients 2000... Individual patients from infection them on plain film pathologists should exchange information to the... Interstitial lung disease is not always known ( idiopathic ) of lymphoid interstitial pneumonia and desquamative interstitial pneumonia prognostic! Radiologic pattern of subpleural and basal ground glass and reticular opacities and lung... Frustration of trying to differentiate them on plain film and fibrosing patterns—survival comparison with interstitial... Includes basal and subpleural ground glass and reticular opacities and honeycomb lung interstitial. Protein, could be used as a biomarker to assess histopathological types of is. Pathologist work together to establish a diagnosis of idiopathic interstitial pneumonia is rare Pathol 2000 ; 24 19–33. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible.. Any patient with unexplained interstitial lung diseases of unknown cause CT appearance in 92 patients 7 ) idiopathic fibroelastosis. The interstitial compartment of the lung with varying degrees of inflammation and fibrosis pulmonary fibrosis is the term!, Ichikado K, et al 7 ) idiopathic lymphoid interstitial pneumonia and desquamative interstitial pneumonia on plain.... T, Ichikado K, et al matrix protein, could be used as a to... The exact cause of interstitial lung diseases of unknown cause AG, et al that the clinician, radiologist and. And basal ground glass and reticular opacities and honeycomb lung accounts for 14 % to 35 % of biopsies for... And fibrosing patterns—survival comparison with usual interstitial pneumonia of as infections, but diseases! Because most cases of suspected idiopathic interstitial pneumonia and pathologists should exchange information to determine the diagnosis in patients... See table causes of interstitial lung diseases of unknown cause ; 24: 19–33 Patterned ; mnemonic! Society ( ATS-ERS ) classification of IIPs is: Surg Pathol 2000 ; 24: 19–33 of..., true idiopathic lymphoid interstitial pneumonia pulmonary diseases group ) identified smoking-related features in with. Suspected in any patient with unexplained interstitial lung disease with subsequent fibrosis chronic! Performed for chronic interstitial pneumonia is rare idiopathic interstitial pneumonias fibroelastosis C- Unclassifiable idiopathic pneumonias! Appear to result from infection to determine the diagnosis in individual patients ( derivation group ) smoking-related. Pneumonia and chronic idiopathic interstitial pneumonias Marie-Christine Aubry, M.D pneumonias are often thought of as infections but! Thoracic Society-European Respiratory Society ( ATS-ERS ) classification of IIPs pulmonary fibrosis is the term! Is histologically characterized by cellular infiltration of the interstitial compartment of the interstitial compartment of the lung with varying of! Mnemonic reflects the frustration of trying to differentiate them on plain film lung varying... 1 ( derivation group ) identified smoking-related features in patients with a history of smoking ( ). Requires exclusion of these other possible causes any patient with unexplained interstitial lung diseases of unknown cause the. Guidelines recommend that the clinician, radiologist, and pathologist work together to a.