Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE)

Oyama, Yoshiyuki; Enomoto, Noriyuki; Suzuki, Yuzo; Kono, Masato; Fujisawa, Tomoyuki; Inui, Naoki; Nakamura, Yutaro; Kuroishi, Shigeki; Yokomura, Koshi; Toyoshima, Mikio; Imokawa, Shiro; Oishi, Keiji; Watanabe, Satoshi; Kondo, Kazuo; Baba, Tomohisa; Ogura, Takashi; Ishii, Hiroshi; Watanabe, Kentaro; Nishioka, Yasuhiko; Suda, Takafumi

doi:10.1016/j.rmed.2016.12.013

Cited by 17 publications

(10 citation statements)

References 30 publications

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“…Histologically, the interstitial increase in predominantly elastic fibers is the fundamental pathogenesis of PPFE [10,11]. Oyama et al [23] reported that urinary concentrations of desmosine were significantly higher in PPFE patients than in IPF patients. In the present study, we showed that the serum concentration of LTBP-4 in IPPFE patients was significantly higher than that in healthy controls.…”

Section: Discussionmentioning

confidence: 99%

Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis

Kinoshita

Ikeda

Kushima

et al. 2020

Respiratory Medicine

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Section: Discussionmentioning

confidence: 99%

Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis

Kinoshita

Ikeda

Kushima

et al. 2020

Respiratory Medicine

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“…Elevated serum autoantibodies and rheumatologic markers like rheumatoid factor and myeloperoxidaseantineutrophil cytoplasmic antibody revealed undetermined significance [3,[25][26][27][28]. Increased levels of urinary desmosine have been shown by Oyama et al in a preliminary study including individuals with biopsyproven PPFE compared to idiopathic pulmonary fibrosis, chronic obstructive pulmonary disease and healthy control subjects with a potential utility of this test as a noninvasive diagnostic marker in patients with suspected but unbiopsied cases of PPFE [29]. None of these indicators are in routine clinical use due to their undetermined significance for PPFE.…”

Section: Discussionmentioning

confidence: 99%

Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis

Tetikkurt

Kubat

Kulahci

et al. 2021

Monaldi Arch Chest Dis

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Idiopathic pleuropulmonary fibroelastosis is an extremely rare lung disease characterized by the combination of fibrosis of the visceral pleura and the fibroelastotic changes transcending in the subpleural lung parenchyma that predominantly affects the upper lobes with accompanying volume loss. It is mostly idiopathic while infection, autoimmunity, bone marrow or lung transplantation and genetic predisposition may be associated with the development of PPFE. The disease is exceptionally rare as approximately ninety cases have been reported in the literature currently. A 35-year-old female presented with exertional dyspnea, dry cough and weight loss. Physical examination demonstrated platythorax, suprasternal notch deepening and fine rales over the upper lobes. Blood count, serum biochemistry, autoimmunity and serologic markers for collagen vascular diseases were within normal limits. Arterial blood gases demonstrated a low pO2 (48 mm Hg) and a high pCO2 (54 mm Hg) values. Chest x-ray showed bilateral parenchymal fibrotic lesions, left pneumothorax, bronchiectasis in the middle and pleural thickening in the upper lung zones while HRCT revealed bilateral apical pleural thickening, traction bronchiectasis, subpleural reticulations, ground-glass opacities and honeycombing in the upper lobes. Bronchoscopy, BAL cytology, smear and culture did not reveal any pathologic findings. Relevant with the clinical, laboratory, radiologic manifestations and the differential diagnosis with other interstitial lung diseases, PPFE was the final diagnosis. The aim of this case report was to present the clinical manifestations of our case. The second crucial objective was to establish a diagnostic scoring system relevant with the literature and the clinical manifestations of the patient.

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“…patients with idiopathic pulmonary fibrosis, chronic obstructive pulmonary disease, and healthy control subjects revealing the potential utility of this test as a non-invasive diagnostic marker in suspected PPFE cases (26). KL-6 and SP-D may be elevated inconsistently in PPFE with unclear consequence while high levels of rheumatologic markers may exist without any significance (6,27,28).…”

Section: Oyama Et Al Have Shown Increased Levels Of Urinary Desmosine...mentioning

confidence: 98%

Diagnosis of pleuroparenchymal fibroelastosis: A review

Tetikkurt

Öztürk²,

Güngördü³

2022

Monaldi Arch Chest Dis

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Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease with unprecedented features characterized by fibroelastotic changes in the subpleural lung parenchyma affecting the upper lobes. PPFE is usually idiopathic, but it can be caused by infection, autoimmunity, bone marrow or lung transplantation, or a genetic predisposition. Histopathologic examination of lung biopsy samples reveals homogenous subpleural fibrosis and abundant elastic fibers, allowing for a definitive diagnosis. As PPFE mimics many interstitial lung diseases, clinicians face significant difficulties in making a definitive final diagnosis. Since most disease-related comorbid conditions manifest at an advanced stage, invasive tissue sampling for histopathologic evaluation is consistently impossible. Such a patient presentation highlights the importance of an analysis based solely on clinical findings, which would provide a definitive diagnosis without the need for a biopsy. Because of its exceptional and inconceivable presentation, PPFE creates a diagnostic dilemma. In light of our two cases and the literature data, we present a diagnostic assessment score assay that relies solely on clinical manifestations without histopathological tissue verification to shed light on the diagnosis of PPFE. This review focuses on PPFE identification through the use of a diagnostic assessment analysis to improve early disease recognition without the use of invasive diagnostic interventions to obtain biopsy samples for histopathologic evaluation. This analytic approach, while not diagnostic in and of itself, may provide a useful pathway for differential diagnosis and may preclude redundant initiatives.

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Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE)

Abstract: Urinary desmosines may be a useful diagnostic biomarker in patients with PPFE. Measurement of desmosines combined with specific clinical and radiological features of PPFE may lead to an accurate diagnosis without SLB in patients with PPFE.

Cited by 17 publications

References 30 publications

Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis

Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis

Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis

Diagnosis of pleuroparenchymal fibroelastosis: A review

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