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PC-US-130481 (03/2023)
Copyright © 2023, Boehringer Ingelheim Pharmaceuticals, Inc. All rights reserved.
03/2023 PC-US-129122
Important information for understanding, diagnosing, and managing ILD
Overview of basic HRCT technique and reconstruction planes
Identification of comorbid conditions on HRCT
Checklists, brochures, guidelines, algorithms, and more valuable resources
acute interstitial pneumonia
(AIP, also known as Hamman-Rich syndrome)
AIP is an acute, idiopathic, and rapidly progressive disease of the lung often leading to fulminant respiratory failure and acute respiratory distress syndrome.1 It can be distinguished clinically from other types of interstitial pneumonia by the rapid onset of respiratory failure in a patient without preexisting lung disease.2 AIP is classified by the American Thoracic Society and European Respiratory Society under major idiopathic interstitial pneumonia and compared to other rare or unclassifiable idiopathic interstitial pneumonia.1
Mrad A, Huda N. Acute interstitial pneumonia. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. PMID: 32119316.
cryptogenic organizing pneumonia (COP)
Organizing pneumonia (OP) is a type of inflammatory reaction of the lung resulting in a pathological syndrome displaying a pattern of intra-alveolar buds of granulation consisting of fibroblasts and myofibroblasts mixed with loose connective matrix. If OP is the result of an underlying disorder (eg, infection, drug toxicity, or connective tissue disease), it is termed secondary OP. When no cause is established, it is termed cryptogenic organizing pneumonia (COP).3
Cottin V, Cordier JF. Cryptogenic organizing pneumonia. Semin Respir Crit Care Med. 2012;33:462-475. doi: http://dx.doi.org/10.1055/s-0032-1325157.
desquamative interstitial pneumonia (DIP)
DIP is a rare type of idiopathic interstitial pneumonia, characterized by large numbers of macrophages in the alveolar spaces and associated with interstitial inflammation and/or fibrosis.4,5 Lymphoid nodules are common along with a sparse but distinct eosinophil infiltrate.5 Most cases of DIP are caused by cigarette smoking, but drugs and other inhaled agents, including marijuana smoke, can also produce the same disease.4,5
Tazelaar HD, Wright JL, Churg A. Desquamative interstitial pneumonia. Histopathology. 2011;58(4):509-16. doi: 10.1111/j.1365-2559.2010.03649.x. Epub 2010 Sep 21. PMID: 20854463.
hypersensitivity pneumonitis (HP, also known as extrinsic allergic alveolitis)
HP is a complex pulmonary syndrome mediated by the immune system and caused by inhalation of a wide variety of antigens in patients previously sensitized.6 The pathobiology of the disease is not fully understood, but in addition to the triggers that initiate the disease, host/genetic factors are thought to be important, as only a minority of exposed individuals develop HP. Acute forms generally resolve without sequelae, while chronic forms, which are caused by persistent low-grade exposures, are associated with poor prognosis.7
Spagnolo P, Rossi G, Cavazza A, et al. Hypersensitivity pneumonitis: a comprehensive review. J Investig Allergol Clin Immunol. 2015;25(4):237-50. PMID: 26310038.
idiopathic non-specific interstitial pneumonia (iNSIP)
NSIP suggested by HRCT or identified on lung pathology is often the result of connective tissue disease, drug toxicity, HP or other causes, but when a cause cannot be attributed to the NSIP pattern it may be classified as iNSIP. Patients with iNSIP typically present in mid-adulthood with dyspnea, cough, and often fever and fatigue. The disease has a female predominance, and is more frequently diagnosed in never-smoker patients. It is highly important to complete a thorough history and assessment for other causes to rule out idiopathic NSIP.8 Multidisciplinary discussion (MDD) is especially important to establish the diagnosis of iNSIP.8
Belloli EA, Beckford R, Hadley R, Flaherty KR. Idiopathic non-specific interstitial pneumonia. Respirology. 2016;21(2):259-68. doi: 10.1111/resp.12674. Epub 2015 Nov 13. PMID: 26564810.
idiopathic pulmonary fibrosis (IPF)
IPF is a progressive and fatal lung disease with increasing prevalence and a median survival of 3–5 years after diagnosis. IPF is characterized by altered cellular composition and dysfunction of resident and immune cells in the lung, leading to an excess of extracellular matrix formation and progressive fibrosis.9
Podolanczuk AJ, Wong AW, Saito S, Lasky JA, Ryerson CJ, Eickelberg O. Update in interstitial lung disease 2020. Am J Respir Crit Care Med. 2021;203(11):1343-1352. doi: 10.1164/rccm.202103-0559UP. PMID: 33835899; PMCID: PMC8759209.
interstitial lung disease (ILD)
ILDs are a heterogeneous group of more than 200 conditions characterized by varying degrees of fibrotic or inflammatory abnormalities of the lung, or a combination of the two.10 ILDs are caused by both known and unknown factors. ILDs can be acute or chronic and may be self-limiting or progressive.10
Yioe V, Phillips G, Spencer LG. Interstitial lung disease on the acute take for the non-respiratory physician. Clin Med (Lond). 2021;21(6):e584-e590. doi: 10.7861/clinmed.2021-0694. PMID: 34862217; PMCID: PMC8806283.
lymphangioleiomyomatosis (LAM)
LAM is a rare disease almost exclusively affecting women of childbearing age.11,12 LAM is a systemic disease with the primary manifestations being pulmonary. It is characterized by the presence of abnormal smooth muscle cells (LAM cells) that infiltrate the lungs and airways, leading to lung cysts and pneumothoraces.12
Cohen MM, Pollock-BarZiv S, Johnson SR. Emerging clinical picture of lymphangioleiomyomatosis. Thorax. 2005;60(10):875-9. doi: 10.1136/thx.2004.035154. Epub 2005 Jul 29. PMID: 16055626; PMCID: PMC1747203.
lymphoid interstitial pneumonia (LIP, also known as lymphocytic interstitial pneumonia)
LIP is a rare interstitial lung disease frequently associated with connective tissue diseases or infections.13 LIP presents with cough, progressive dyspnea, and crackles.14,15 Idiopathic LIP is rare, so every attempt must be made to diagnose underlying conditions when LIP is diagnosed.15 Demonstrating polyclonality with immunohistochemistry is the key to differentiating LIP from lymphoma.13 The 5-year mortality remains as high as 50% and is likely to vary based on the underlying disease process.14
Panchabhai TS, Farver C, Highland KB. Lymphocytic interstitial pneumonia. Clin Chest Med. 2016;37(3):463-74. doi: 10.1016/j.ccm.2016.04.009. Epub 2016 Jun 25. PMID: 27514593.
pleuroparenchymal fibroelastosis (PPFE)
PPFE is a rare idiopathic interstitial pneumonia with unique clinical, radiological, and pathological characteristics. PPFE presents with a combination of fibrosis involving the visceral pleura and fibroelastotic changes predominating in the subpleural lung parenchyma. While PPFE has been associated with a number of diseases, no single cause has been identified.16
Chua F, Desai SR, Nicholson AG, et al. Pleuroparenchymal fibroelastosis. A review of clinical, radiological, and pathological characteristics. Ann Am Thorac Soc. 2019;16(11):1351-1359. doi: 10.1513/AnnalsATS.201902-181CME. PMID: 31425665; PMCID: PMC6945468.
systemic sclerosis (SSc)
Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by fibrosis, and by vascular and immunological abnormalities. SSc is classified based on skin involvement and there are 2 main subtypes of SSc, limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc). LcSSc is associated with skin thickening distal to the elbows, distal to the knees, and/or on the face without trunk involvement. DcSSc is associated with skin thickening proximal to the knees, on the face, and/or the trunk. Both subtypes can affect the lungs, as well as the gastrointestinal tract, kidneys, skeletal muscle, and pericardium.17
Adigun R, Goyal A, Hariz A. Systemic Sclerosis. 2022 May 8. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. PMID:28613625.
Overview of basic HRCT technique and reconstruction planes
Identification of comorbid conditions on HRCT
Radiology Checklist Tool
of patients with ILD had at least 1 misdiagnosis
it can take 2 or more years to diagnose
diagnosis sometimes requires 3 or more different doctors
Reference:
1. Cosgrove GP et al. BMC Pulm Med. 2018;18(1):9.
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