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GLOSSARY & ABBREVIATIONS

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.

ACE, angiotensin-converting enzyme
AIP, acute interstitial pneumonia
ANA, antinuclear antibody
ATA, anti-topoisomerase antibodies
BAL, bronchoalveolar lavage
CCP, cyclic citrullinated peptide
CHF, congestive heart failure
COP, cryptogenic organizing pneumonia
COPD, chronic obstructive pulmonary disease
CT, computed tomography
CTD, connective tissue disease
DIP, desquamative interstitial pneumonia
DL_CO, diffusing capacity of the lungs for carbon monoxide
dcSSc, diffuse cutaneous systemic sclerosis
FEV₁, forced expiratory volume in one second
FVC, forced vital capacity
GERD, gastroesophageal reflux disease
HLA, human leukocyte antigen
HP, hypersensitivity pneumonitis
HRCT, high-resolution computed tomography
HTN, hypertension
ILD, interstitial lung disease
iNSIP, idiopathic non-specific interstitial pneumonia
IPF, idiopathic pulmonary fibrosis
LAM, lymphangioleiomyomatosis
LIP, lymphoid interstitial pneumonia
MDD, multidisciplinary discussion
MI, myocardial infarction
NSIP, non-specific interstitial pneumonia
OP, organizing pneumonia
PFT, pulmonary function test
PPFE, pleuroparenchymal fibroelastosis
RA-ILD, rheumatoid arthritis-associated interstitial lung disease
RB-ILD, respiratory bronchiolitis-associated interstitial lung disease
RF, rheumatoid factor
RV, residual volume
Scl-70, scleroderma-70 (topoisomerase I)
SLB, surgical lung biopsy
SpO₂, oxygen saturation
SS-A, Sjögren’s syndrome-A (anti-Ro)
SS-B, Sjögren’s syndrome-B (anti-La)
SSc, systemic sclerosis
SSc-ILD, systemic sclerosis-associated interstitial lung disease
TBLC, transbronchial lung cryobiopsy
TLC, total lung capacity
TPO, thyroid peroxidase
uILD, unclassifiable interstitial lung disease
UIP, usual interstitial pneumonia
VATS, video-assisted thoracoscopic surgery

References:
1. Travis WD et al. Am J Respir Crit Care Med. 2013;188(6):733-748. 2. Bouros D et al. Eur Respir J. 2000;15:412-418. 3. Cottin V et al. Semin Respir Crit Care Med. 2012;33:462-475. 4. Godbert B et al. Eur Resp Rev. 2013;22(128):117-123. 5. Tazelaar HD et al. Histopathology. 2011;58(4):509-516. 6. Costabel U et al. Clin Chest Med. 2012;33(3):151-163. 7. Spagnolo P et al. J lnvestig Allergol Clin lmmunol. 2015;25(4):237-250. 8. Belloli EA et al. Respirology. 2016;21(2):259-268 9. Podolanczuk AJ et al. 2020. Am J Respir Crit Care Med. 2021;203(11):1343-1352. 10. Yioe V et al. Clin Med. 2021;21(6):e584-e590. 11. Cohen MM et al. Thorax. 2005;60(10):875-879. 12. Wakida K et al. Ann Thorac Surg. 2015;100:1105-1107. 13. Panchabhai T et al. Clin Chest Med. 2016;37(3):463-474. 14. Swigris J et al. Chest. 2002;122(6):2150-2164. 15. Cha S-I et al. Eur Respir J. 2006:28:364-369. 16. Chua F et al. Ann Am Thorac Soc. 2019;16(11):1351-1359. 17. Adigun R et al. Systemic Sclerosis. 2022 May 8. In: StatPearls [Internet]. 2022 Jan-. PMID: 28613625.

INTRODUCTION

Up to 246,000 people in the US are estimated to have an ILD

IPF is one of the most common ILDs³

IPF is estimated to affect about 140,000 people in the US. 3-5 years median survival.

Risk factors in patients with IPF⁵

Male gender
Older age
History of smoking
Microbial agents
Environmental exposures
Family history

ILD is a common, often early, and potentially fatal manifestation in autoimmune diseases⁶

Up to 30% of patients with RA develop ILD. 39% of patients with RA-ILD may die within 5 years.

Risk factors in patients with RA

4x greater risk in males vs females¹⁰
Typically occurs in people 40-50 years of age¹¹
History of smoking¹¹
Rheumatoid factor or anti-CCP antibody¹¹

ILD is a common, often early, and potentially fatal manifestation in autoimmune diseases⁶

Up to 80% of patients with SSc develop ILD. 18% of patients with SSc-ILD may die within 5 years.

Risk factors in patients with SSc

Older age¹⁵
Male gender¹⁵
Short disease duration¹⁶
Diagnosis of dcSSc¹⁷
Presence of ATA¹⁸

*Estimate calculated based on overall ILD prevalence of 74.3 cases per 100,000 in the US, and an overall run-up population of 330.5 million in the US.^1,2

References:
1. Wijsenbeek M, Cottin V. N Engl J Med. 2020;383:958-968. 2. Monthly Population Estimates for the United States: April 1, 2020 to December 1, 2022 (NA-EST2021-POP). US Census Bureau, Population Division; 2021. 3. Oldham JM, Noth I. Respir Med. 2014;108(6):819-829. 4. Raghu G et al. Am J Respir Crit Care Med. 2006;174(7):810-816. 5. Raghu G et al; on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2011;183(6):788-824. 6. Fischer A, Distler J. Clin Rheumatol. 2019;38(10):2673-2681. 7. Geerts S et al. Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(4):326-335. 8. Shaw M et al. Eur Respir Rev. 2015;24(135):1-16. 9. Hyldgaard C et al. Respirology. 2017;22(3):494-500. 10. Esposito AJ et al. Clin Chest Med. 2019;40(3):545-560. 11. Koo SM et al. Tuberc Respir Dis (Seoul). 2019;82(4):285-297. 12. Fischer A et al. Arthritis Rheumatol. 2019;71(2):182-195. 13. Khanna D et al. Am J Respir Crit Care. 2020;201(6):650-660. 14. Hoffmann-Vold AM et al. Am J Respir Crit Care Med. 2019;200(10):1258-1266. 15. Cappelli S et al. Eur Respir Rev. 2015;24(137):411-419. 16. Steen V. Ann Rheum Dis. 2003;62(2):97-99. 17. Walker UA et al. Ann Rheum Dis. 2007;66(6):754-763. 18. Assassi S et al. Arthritis Res Ther. 2010;12(5):R166.