Krebs Von Den Lungen-6 (KL-6) Is A Useful Biomarker For Life-Threatening EGFR-TKI Related Interstitial Lung Disease

Author(s):  
Shigeo Kawase ◽  
Noboru Hattori ◽  
Nobuhisa Ishikawa ◽  
Yasushi Horimasu ◽  
Osamu Furonaka ◽  
...  
2021 ◽  
Vol 11 (2) ◽  
pp. 235-240
Author(s):  
Houari Aissaoui ◽  
Kinan Drak Alsibai ◽  
Naji Khayath

Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management.


Rheumatology ◽  
2018 ◽  
Vol 57 (9) ◽  
pp. 1688-1689 ◽  
Author(s):  
Marion Delplanque ◽  
Marc Gatfosse ◽  
Hafid Ait-Oufella ◽  
Olaf Mercier ◽  
Laurent Savale ◽  
...  

2011 ◽  
Vol 12 (1) ◽  
Author(s):  
Shigeo Kawase ◽  
Noboru Hattori ◽  
Nobuhisa Ishikawa ◽  
Yasushi Horimasu ◽  
Kazunori Fujitaka ◽  
...  

2008 ◽  
Vol 113 (3) ◽  
pp. 609-612 ◽  
Author(s):  
M. J. Pepels ◽  
K. A. Boomars ◽  
R. van Kimmenade ◽  
P. S. Hupperets

2019 ◽  
Author(s):  
Teppei Yamaguchi ◽  
Junichi Shimizu ◽  
Takaaki Hasegawa ◽  
Yoshitsugu Horio ◽  
Yoshitaka Inaba ◽  
...  

Abstract BackgroundNivolumab, an anti-programmed death 1 (PD-1) monoclonal antibody, has shown survival benefit in clinical trials of various malignant tumors. Nivolumab-induced pneumonitis is major immune-related adverse event (irAE) that is occasionally serious and life-threatening. The aim of this study was to examine the association between pre-existing interstitial lung disease (ILD) on chest computed tomography (CT) and nivolumab-induced pneumonitis among different types of solid tumors.MethodsWe retrospectively collected the clinical data of 311 patients who were diagnosed with non-small cell lung cancer (NSCLC), head and neck cancer (HNC), or gastric cancer (GC), and treated with nivolumab monotherapy. Patients who underwent chest CT immediately before starting nivolumab without previous thoracic radiotherapy or other immune checkpoint inhibitors were eligible. We collected baseline patient characteristics and assessed pre-existing ILD on baseline chest CT.ResultsFinally, 188 patients were included in the analysis: 96 patients with NSCLC, 43 patients with HNC, and 49 patients with GC. NSCLC patients had a significantly higher rate of pre-existing ILD compared with HNC/GC patients (P=0.047). Nivolumab-induced pneumonitis occurred in 11.7% (22 of 188), including 14.6% (14 of 96) of NSCLC, and 8.7% (8 of 92) of HNC/GC. Univariate and multivariate logistic regression analyses revealed that pre-existing ILD (odds ratio, 5.92; 95% confidence interval (CI), 2.07–18.54, P=0.0008) and male sex (odds ratio, 5.58; 95% CI, 1.01–104.40, P=0.049) significantly increased the risk of nivolumab-induced pneumonitis.ConclusionOur results indicated that pre-existing ILD and male sex are risk factors for nivolumab-induced pneumonitis in solid tumors.


Thorax ◽  
2019 ◽  
Vol 75 (1) ◽  
pp. 92-95 ◽  
Author(s):  
Marie-Louise Frémond ◽  
Marie Legendre ◽  
Michael Fayon ◽  
Annick Clement ◽  
Emilie Filhol-Blin ◽  
...  

COPA (coatomer subunit α) syndrome is a newly recognised cause of interstitial lung disease in children and adults, frequently associated with arthritis and renal dysfunction. We report a 11-year-old girl with disease limited to major pulmonary haemosiderosis manifesting at the age of 2 years, due to a heterozygous p.(Arg233His) mutation in COPA. Her interferon (IFN) signature was elevated (10.312 and 12.429, healthy <2.466), as was the level of serum IFNα (211 fg/mL, healthy <10 fg/mL). STAT1 phosphorylation in T lymphocytes and monocytes was increased as compared with healthy controls. Based on these results she was treated with the JAK1/2 inhibitor ruxolitinib, which resulted in reduction in IFN signalling and appeared to be associated with partial though incomplete decrease in the severity of her pulmonary disease. Patients with alveolar haemorrhage of unknown origin should be considered for COPA screening. Functional tests can help to personalise patient therapy.


2013 ◽  
Vol 43 (4) ◽  
pp. 422-425 ◽  
Author(s):  
Shinji Nakamichi ◽  
Kaoru Kubota ◽  
Hidehito Horinouchi ◽  
Shintaro Kanda ◽  
Yutaka Fujiwara ◽  
...  

2020 ◽  
Vol 6 (2) ◽  
pp. 00306-2019
Author(s):  
Shohei Hamada ◽  
Hidenori Ichiyasu ◽  
Megumi Inaba ◽  
Hiroshi Takahashi ◽  
Tomoki Sadamatsu ◽  
...  

BackgroundThe increasing incidence of life-threatening Pneumocystis pneumonia (PCP) in non-HIV immunocompromised patients is a global concern. Yet, no reports have examined the prognostic significance of pre-existing interstitial lung disease (ILD) in non-HIV PCP.MethodsWe retrospectively reviewed the medical records of non-HIV PCP patients with (ILD group) or without (non-ILD group) pre-existing ILD. The clinical features and outcomes of the ILD group were compared with those of the non-ILD group. Cox regression models were constructed to identify prognostic factors.Results74 patients were enrolled in this study. The 90-day mortality was significantly higher in the ILD group than in the non-ILD group (62.5% versus 19.0%, p<0.001). In the ILD group, patients with a higher percentage of bronchoalveolar lavage fluid neutrophils had worse outcomes compared to those having a lower percentage (p=0.026). Multivariate analyses revealed that pre-existing ILD (p=0.002) and low levels of serum albumin (p=0.009) were independent risk factors for 90-day mortality. Serum levels of β-d-glucan were significantly reduced after treatment of PCP in both groups, whereas levels of Krebs von den Lungen-6 (KL-6) significantly increased in the ILD group. In the ILD group, the 90-day mortality of patients with increasing KL-6 levels after treatment was significantly higher than those with decreasing levels (78.9% versus 0%, p=0.019).ConclusionIn non-HIV PCP patients, pre-existing ILD is associated with a poorer prognosis. Prophylaxis for PCP is needed in patients with pre-existing ILD under immunosuppression.


2019 ◽  
Vol 53 (6) ◽  
pp. 492-496
Author(s):  
Yuan Li ◽  
Guang-Chao Gu ◽  
Bao Liu ◽  
Jiang Shao ◽  
Yu Chen ◽  
...  

Bronchial artery aneurysm (BAA) is a rare entity. Ruptured BAA can cause life-threatening hemorrhage. It is recommended that treatment should be initiated immediately after diagnosis. We present the case of a 56-year-old female with multiple BAAs and interstitial lung disease. Aortic computed tomography angiography demonstrated that the largest aneurysm at the right hilum was fed by right subclavian artery and right bronchial artery. A fistula between the pulmonary trunk and the aneurysm was also revealed. The patient underwent transcatheter embolization. Coils were placed in the feeding vessels instead of the aneurysms to avoid nontarget embolization of the pulmonary arteries through the fistula. The procedure achieved reduction in aneurysmal blood flow. The patient’s cough resolved at 6-month follow-up.


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