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Idiopathic pulmonary fibrosis: Current and future treatment

Glass, Daniel S; Grossfeld, David; Renna, Heather A; Agarwala, Priya; Spiegler, Peter; DeLeon, Joshua; Reiss, Allison B
OBJECTIVES/OBJECTIVE:Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea. Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads inexorably to respiratory failure and death within 3-5 years of diagnosis. This review discusses treatment approaches to IPF in current use and those that appear promising for future development. DATA SOURCE/METHODS:The data were obtained from the Randomized Controlled Trials and scientific studies published in English literature. We used search terms related to IPF, antifibrotic treatment, lung transplant, and management. RESULTS:Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells. The mainstay therapies are the oral antifibrotic drugs pirfenidone and nintedanib, which can improve quality of life, attenuate symptoms, and slow disease progression. Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy. CONCLUSION/CONCLUSIONS:Clearly, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving well-being.
PMID: 35001525
ISSN: 1752-699x
CID: 5175912

Idiopathic pulmonary fibrosis: Molecular mechanisms and potential treatment approaches

Glass, Daniel S; Grossfeld, David; Renna, Heather A; Agarwala, Priya; Spiegler, Peter; Kasselman, Lora J; Glass, Amy D; DeLeon, Joshua; Reiss, Allison B
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease with high mortality that commonly occurs in middle-aged and older adults. IPF, characterized by a decline in lung function, often manifests as exertional dyspnea and cough. Symptoms result from a fibrotic process driven by alveolar epithelial cells that leads to increased migration, proliferation, and differentiation of lung fibroblasts. Ultimately, the differentiation of fibroblasts into myofibroblasts, which synthesize excessive amounts of extracellular matrix proteins, destroys the lung architecture. However, the factors that induce the fibrotic process are unclear. Diagnosis can be a difficult process; the gold standard for diagnosis is the multidisciplinary conference. Practical biomarkers are needed to improve diagnostic and prognostic accuracy. High-resolution computed tomography typically shows interstitial pneumonia with basal and peripheral honeycombing. Gas exchange and diffusion capacity are impaired. Treatments are limited, although the anti-fibrotic drugs pirfenidone and nintedanib can slow the progression of the disease. Lung transplantation is often contraindicated because of age and comorbidities, but it improves survival when successful. The incidence and prevalence of IPF has been increasing and there is an urgent need for improved therapies. This review covers the detailed cellular and molecular mechanisms underlying IPF progression as well as current treatments and cutting-edge research into new therapeutic targets.
PMID: 32487481
ISSN: 2212-5353
CID: 4468982

Six-Minute Walk Test: Clinical Role, Technique, Coding and Reimbursement

Agarwala, Priya; Salzman, Steve H
PMID: 31689414
ISSN: 1931-3543
CID: 4172612


Pletukhina, Nadia; Kasselman, Lora J.; Salzman, Steven H.; Renna, Heather A.; Reiss, Allison B.; Agarwala, Priya
ISSN: 1081-5589
CID: 3049432

Variability in FEV1 in Comparison to Forced Vital Capacity in Patients with Idiopathic Pulmonary Fibrosis [Meeting Abstract]

Ashraf, S.; Wong, K. A.; Agarwala, P.; Salzman, S. H.
ISSN: 1073-449x
CID: 3509632

Heme oxygenase-1-dependent central cardiorespiratory adaptations to chronic intermittent hypoxia in mice

Sunderram, Jag; Semmlow, John; Patel, Pranav; Rao, Harshit; Chun, Glen; Agarwala, Priya; Bhaumik, Mantu; Le-Hoang, Oanh; Lu, Shou-En; Neubauer, Judith A
Chronic intermittent hypoxia (CIH) increases sympathetic tone and respiratory instability. Our previous work showed that chronic hypoxia induces the oxygen-sensing enzyme heme oxygenase-1 (HO-1) within the C1 sympathoexcitatory region and the pre-Bötzinger complex (pre-BötC). We therefore examined the effect of CIH on time course of induced expression of HO-1 within these regions and determined whether the induction of HO-1 correlated with changes in respiratory, sigh frequency, and sympathetic responses (spectral analysis of heart rate) to acute hypoxia (10% O2) during 10 days of exposure to CIH in chronically instrumented awake wild-type (WT) and HO-1 null mice (HO-1-/-). HO-1 was induced within the C1 and pre-BötC regions after 1 day of CIH. There were no significant differences in the baseline respiratory parameters between WT and HO-1-/- Prior to CIH, acute hypoxia increased respiratory frequency in both WT and HO-1-/-; however, minute diaphragm electromyogram activity increased in WT but not HO-1-/- The hypoxic respiratory response after 1 and 10 days of CIH was restored in HO-1-/- CIH resulted in an initial significant decline in 1) the hypoxic sigh frequency response, which was restored in WT but not HO-1-/-, and 2) the baseline sympathetic activity in WT and HO-1-/-, which remained stable subsequently in WT but not in HO-1-/- We conclude that 1) CIH induces expression of HO-1 in the C1 and pre-BötC regions within 1 day and 2) HO-1 is necessary for hypoxia respiratory response and contributes to the maintenance of the hypoxic sigh responses and baseline sympathetic activity during CIH.
PMID: 27609199
ISSN: 1522-1601
CID: 3428592

The Role Of Nasal Resistance In Cpap Adherence In The World Trade Center First Responder Cohort [Meeting Abstract]

Agarwala, P.; Ducca, E.; Gumb, T.; Twumasi, A.; Black, K.; Lewis, C.; Alimokhtari, S.; Perez, A.; Lu, S. -E.; Chitkara, N.; Harrison, D.; Udasin, I.; Sunderram, J.; Rapoport, D.; Ayappa, I. A.
ISSN: 1073-449x
CID: 3428712

Reactive Airways After Peracetic Acid Inhalation Following A Home Brewing Accident [Meeting Abstract]

Agarwala, P.; Lubinsky, A. S.
ISSN: 1073-449x
CID: 3428702

Nasal Inflammation Is Associated With Chronic RhINOSinusitis (crs) And Obstructive Sleep Apnea (osa) In World Trade Center Responders: Preliminary Results From The Wtc Snore Study [Meeting Abstract]

Sunderram, J; Plietz, M; Ayappa, IA; Laumbach, RJ; Lu, S-E; Black, K; Alimokhtari, S; Perez, A; Le-Hoang, O; Kipen, HM; Carson, JL; Udasin, I; Twumasi, A; Agarwala, P; Gumb, T; Chitkara, N; Harrison, D; Rapoport, DM
ISSN: 1535-4970
CID: 2162132

Relationship Of Chronic RhINOSinusitis And Osa In Wtc Responders: Preliminary Results From The Wtc Snore Study [Meeting Abstract]

Sunderram, J.; Ayappa, I. A.; Vakil, J.; Patel, R.; Agarwala, P.; Cepeda, C.; Twumasi, A.; Black, K.; Chitkara, N.; Laumbach, R. J.; Lu, S. -E.; Kipen, H. M.; Carson, J. L.; Harrison, D.; Udasin, I.; Rapoport, D. M.
ISSN: 1073-449x
CID: 2960142