Interstitial Lung Disease: 150 Years of Progress

Joshua J. Solomon, MD, Editor

Kevin K. Brown, MD, Editor

In 1873, von Buhl published one of the earliest histologic descriptions of interstitial lung disease (ILD) in Tuberkulose und Schwindsucht.1Lungenentzündung, Tuberkulose und Schwindsucht: zwö lf Briefe an einen Freund. He described a disorder pathologically characterized by “degeneration and desquamation of alveolar and bronchiolar epithelium” likely caused by tuberculosis or syphilis and coined the term “chronic interstitial pneumonia.” The first histologic description of what today we call idiopathic pulmonary fibrosis (IPF) was published in 1897 by Rindfleisch, calling the new entity “Cirrhosis Cystica Pulmonum.2Ueber cirrhosis cystica pulmonum.” In 1912, von Hansemann described cases of organizing pneumonia secondary to tuberculosis and suggested the term “Lymphangitis Reticularis Pulmonum.3Die Lymphangitis reticularis der Lungen als selbstandige Erkrankung.” In the early 1930s, Hamman and Rich described a rapidly progressive ILD that they called “Acute Diffuse Interstitial Fibrosis of the Lungs,” later colloquially termed “Hamman-Rich Syndrome.4Fulminating diffuse interstitial fibrosis of the lungs.” This description was an early step toward the recognition of IPF as a distinct clinical entity with a high mortality. Dr Averill Liebow was the first to recognize the distinct pathologic pattern of fibrosis in IPF, using the term “Usual Interstitial Pneumonia” to reflect that this was the most common pattern of fibrosis seen in his practice. In the mid-1960s, Liebow and Carrington extended these findings by describing and naming five common histopathologic patterns of ILD and argued that the pattern suggested cause.5Liebow A.A. Carrington C.B. The interstitial pneumonias, frontiers of pulmonary radiology. These observations set the stage for the first international consensus on ILD, summarized by the 2000 American Thoracic Society and European Respiratory Society statement on the diagnosis and treatment of IPF.

These humble beginnings have prepared us for our current era of understanding, ushered in by advances in omics (genomics, epigenomics, transcriptomics, proteomics, and metabolomics), and a rapidly expanding era of drug development. The first effective medications for IPF were approved in 2014, and we now have over 20 promising drugs in phase 2 or 3 trials.

As we have made progress, in this issue of Immunology and Allergy Clinics of North America on “Interstitial Lung Disease,” we summarize our current understanding of both the common and the uncommon disorders. Drs Strykowski and Adegunsoye update us on the most common and lethal of the ILDs, IPF. In addition, they review progressive pulmonary fibrosis, a new concept that groups together ILDs based on shared pathologic features, longitudinal behavior, and a poor prognosis. Drs Matson and Demoruelle help us navigate the ever-growing field of connective tissue disease–associated ILD (CTD-ILD) with a review of both the clinical features and the potential pathogenic pathways. Dr Pandya and her colleagues skillfully outline the complexities of the diagnosis and management of hypersensitivity pneumonitis (HP), a heterogenous disease that can mimic others and one where management involves more than just pharmacotherapy. In the words of Drs Spagnolo and Nicol Bernardinello, sarcoidosis remains a diagnostic challenge by virtue of its “complex multidimensional nature…coupled with its wide range of clinical manifestations,” and their review helps us navigate the diagnosis and management of the multiorgan system involvement. The spectrum of smoking-related ILDs is nicely reviewed by Dr Alarcon-Calderon and colleagues, and Dr Cottin provides us with a thorough review of the wide range of pulmonary diseases associated with eosinophils. Drs Glaspole and Barnes’ review on occupational lung disease reminds us that occupational exposures can cause primary disease as well as contribute to the development of more common forms of ILD, such as HP and CTD-ILD. Dr Ng and colleagues ask us to consider drug-induced lung disease in our differential diagnoses and that this diagnosis is fraught with challenges. The next two articles cover less-common ILDs; Dr Koslow and colleagues cover the range of rare cystic lung diseases and provide us with an algorithmic approach to their diagnosis. ILD associated with anti-neutrophil cytoplasm antibody (ANCA) vasculitis is a challenge both diagnostically and therapeutically; Dr Stewards and colleagues review our current understanding of this rare but serious ILD. The recent pandemic has provided us with a potentially new disorder to add to our list—COVID-induced ILD. Dr Kewalrammani and colleagues take us through this developing body of literature. In our final article, Dr Pugashetti and colleagues provide us with a thorough review of the ever-growing list of biomarkers in the world of CTD-ILD.

We recognize the outstanding combined efforts of these authors and trust that this issue of Immunology and Allergy Clinics of North America will give you an appreciation of the breadth and complexity of ILD; insight into their clinical presentations, diagnostic pathways, and management; as well as a glimpse into the future.

References

Lungenentzündung, Tuberkulose und Schwindsucht: zwö lf Briefe an einen Freund.

Bayrische Staatsbibliothek München, Oldenbourg

Ueber cirrhosis cystica pulmonum.

Zentralbl Pathol. 8: 864-865

Die Lymphangitis reticularis der Lungen als selbstandige Erkrankung.

ViIrhows Arch [Pathol Anaq. 220: 311-321

Fulminating diffuse interstitial fibrosis of the lungs.

Trans Am Clin Climatol Assoc. 51: 154-163Liebow A.A. Carrington C.B.

The interstitial pneumonias, frontiers of pulmonary radiology.

Grune and Stratton, New YorkArticle infoIdentification

DOI: https://doi.org/10.1016/j.iac.2023.03.001