A patient with pleuroparenchymal fibroelastosis carrying a novel fibrillin-2 gene variant.

Pleuroparenchymal fibroelastosis is a recently recognized clinical entity characterized by interstitial pneumonia with proliferating elastin in the upper lung regions. Pleuroparenchymal fibroelastosis is categorized as idiopathic or reported depending on the coexistent initiating factors; however, congenital contractural arachnodactyly, which is caused by abnormal production of elastin based on a mutation in the fibrillin-2 gene, is rarely reported with lung lesion resembling pleuroparenchymal fibroelastosis. We present a case of pleuroparenchymal fibroelastosis in a patient with a novel mutation in the fibrillin-2 gene, which encodes the prenatal fibrillin-2 protein as a scaffold for elastin.

Association between increased nonaerated lung weight and treatment failure in patients with de novo acute respiratory failure: Difference between high-flow nasal oxygen therapy and noninvasive ventilation in a multicentre retrospective study.

PURPOSE: To determine the association between lung collapse and treatment failure in high-flow nasal cannula oxygen therapy (HFNC) or noninvasive ventilation (NIV). METHODS: We performed a quantitative analysis of chest computed tomography in patients with de novo acute respiratory failure who received HFNC (HFNC group) or NIV (NIV group) between January 2012 and December 2017. In the HFNC and NIV group, the nonaerated lung weight were compared between patients in whom respiratory treatment succeeded or failed, respectively. We used logistic regression to examine the association between the nonaerated lung weight and treatment failure. RESULTS: Treatment failed in 70/118 (59%) patients in the HFNC group and 66/101 (65%) patients in the NIV group. The nonaerated lung weight was significantly greater in unsuccessfully treated patients than in successfully treated patients in the HFNC group (p = 0.005), but not in the NIV group (p = 0.535). Logistic regression revealed that greater nonaerated lung weight was associated with increased risk of HFNC failure (adjusted odds ratio 1.30 per 5% increase, 95% confidence interval 1.09-1.55, p = 0.003) but not of NIV failure. CONCLUSIONS: Patients with a greater nonaerated lung weight had a higher risk of HFNC failure, but not of NIV failure.

Development of mesothelioma in situ and its progression to invasive disease observed in a patient with uncontrolled pleural effusions for 15 years.

Mesothelioma in situ (MIS) has recently been investigated as a distinct phase of mesothelioma carcinogenesis. The diagnostic criteria proposed for MIS include a loss of BRCA1-associated protein 1 (BAP1) expression detected by immunohistochemistry (IHC). However, the length of time that MIS typically remains an in situ lesion before progression to invasive disease is still unclear. Herein, we report a case of a Japanese woman in her early seventies who had suffered from recurrent pleural effusions for 15 years, during which MIS developed and progressed to invasive mesothelioma. Retrospective diagnosis of partial MIS, fully developed MIS, and invasive disease was made using BAP1 IHC on three biopsy specimens and via clinical observations with radiological images. MIS and invasive lesions revealed BAP1 loss. The interval from partial or full MIS to invasive disease was 14 and 7 years, respectively. These results support a diagnostic strategy combining histomorphology with genomic-based assays including BAP1 IHC in biopsy tissues from a patient with recurrent pleural effusions.

Comparison of high-flow nasal cannula oxygen therapy and non-invasive ventilation as first-line therapy in respiratory failure: a multicenter retrospective study.

AIM: To identify which subgroups of respiratory failure could benefit more from high-flow nasal cannula oxygen therapy (HFNC) or non-invasive ventilation (NIV). METHODS: We undertook a multicenter retrospective study of patients with acute respiratory failure (ARF) who received HFNC or NIV as first-line respiratory support between January 2012 and December 2017. The adjusted odds ratios (OR) with 95% confidence intervals (CI) for HFNC versus NIV were calculated for treatment failure and 30-day mortality in the overall cohort and in patient subgroups. RESULTS: High-flow nasal cannula oxygen therapy and NIV were used in 200 and 378 patients, and the treatment failure and 30-day mortality rates were 56% and 34% in the HFNC group and 41% and 39% in the NIV group, respectively. The risks of treatment failure and 30-day mortality were not significantly different between the two groups. In subgroup analyses, HFNC was associated with increased risk of treatment failure in patients with cardiogenic pulmonary edema (adjusted OR 6.26; 95% CI, 2.19-17.87; P < 0.01) and hypercapnia (adjusted OR 3.70; 95% CI, 1.34-10.25; P = 0.01), but the 30-day mortality was not significantly different in these subgroups. High-flow nasal cannula oxygen therapy was associated with lower risk of 30-day mortality in patients with pneumonia (adjusted OR 0.43; 95% CI, 0.19-0.94; P = 0.03) and in patients without hypercapnia (adjusted OR 0.51; 95% CI, 0.30-0.88; P = 0.02). CONCLUSION: High-flow nasal cannula oxygen therapy could be more beneficial than NIV in patients with pneumonia or non-hypercapnia, but not in patients with cardiogenic pulmonary edema or hypercapnia.

Case of a fractured human bone fragment as an endobronchial foreign body following a traffic incident.

Endobronchial foreign bodies (EFBs) are present in various settings, such as eating or dental procedure. Accidental aspiration of foreign bodies is more common in children; however, cases of adult foreign body aspiration exist. Traumatic incidents can precipitate endobronchial aspiration of foreign bodies. Loss of consciousness, such as in coma, can result in foreign bodies being easily inhaled into the airways. Teeth or vehicle parts have been reported as EFBs following traumatic incidents. We report on a patient with chronic, sustained cough following maxillofacial trauma. Chest CT revealed an abnormal calcified endobronchial opacity. Flexible bronchoscopy confirmed the presence of an endobronchial foreign body in the left main bronchus. Following removal by bronchial forceps, the body was identified as a human bone fragment. Successful removal of the endobronchial bone fragment resulted in complete symptom remission. We concluded that post-traumatic respiratory complaints should be comprehensively evaluated, even if mild.

Familial diffuse pulmonary ossification: A possible genetic disorder.

Diffuse pulmonary ossification (DPO) is an uncommon disease that is characterized by the widespread formation of ectopic bone in the lungs. Herein, we describe two familial cases of DPO. The patients were a 47-year-old woman and her 76-year-old father. Both patients had a history of recurrent cough, and their chest images showed multiple nodules with ossification. No underlying diseases that might have caused DPO were evident; however, the female patient also had congenital finger hypoplasia and deformity. The present cases indicate the possibility that DPO might be partly associated with genetic disorders.

Two cases of pleuroparenchymal fibroelastosis diagnosed with transbronchial lung biopsy.

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare subset of idiopathic interstitial pneumonias (IIPs). Here we present two patients with PPFE in which the histology was confirmed with transbronchial lung biopsy (TBLB). The 25-year-old and 64-year-old men were both slender with a long history of pulmonary upper lobe fibrosis and a marked restrictive impairment. Although the imaging findings supported the diagnosis of PPFE, surgical lung biopsy (SLB) seemed to be needed to identify fibroelastosis for the definite diagnosis. However, we selected TBLB instead of SLB because of their general condition and the risk such as prolonged pneumothorax after TBLB. TBLB specimens in both patients showed aggregates of elastic fibers in the submucosa that were essential clues for the histological diagnosis of PPFE. TBLB may be an alternative tool for the histological diagnosis of PPFE, although a multidisciplinary discussion is necessary for the final diagnosis of PPFE as a clinicopathological entity.

Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation.

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related, interstitial lung disease, and pulmonary hypertension (PH) is associated with mortality. We herein report a case of PLCH complicated by severe PH and respiratory impairment. After developing PH, the patient displayed a cystic pattern on chest high-resolution computed tomography (HRCT). This, in turn, corresponded with the scarring stage of PLCH. However, the patient's PH and respiratory impairment improve dramatically following smoking cessation. PLCH patients with a cystic pattern on chest HRCT may still be able to improve their PH and respiratory impairment when they are able to quit smoking.

Granulomatous interstitial pneumonia induced by the intake of rice bran pickles: a rare subtype of pulmonary manifestation associated with food allergy.

Pulmonary manifestations associated with food allergy are rich in variety. We report the first case of food-induced granulomatous interstitial pneumonia mimicking hypersensitivity pneumonitis (HP). A 77-year-old woman with respiratory symptoms was referred to our hospital. We performed a surgical lung biopsy, which showed the features of granulomatous interstitial pneumonia. Her clinical history resembled those observed in HP. However, avoidance of exposure to the causative antigens did not improve her symptoms. Moreover, the patient had some features inconsistent with HP, such as elevated serum IgE levels, blood eosinophilia, intrathoracic lymphadenopathies and pleural effusion. Therefore, we pursued another extrinsic non-inhaled antigen as the cause of pulmonary involvements. We noted that she had been eating homemade rice bran pickles, and pulmonary involvements were induced by an ingestion challenge test. We suggest that granulomatous interstitial pneumonia may be a rare subtype of the pulmonary manifestations associated with food allergy.

Recovery of chylous pulmonary congestion in tuberous sclerosis complex-associated lymphangioleiomyomatosis.

Chyloptysis and chylous pulmonary congestion are extremely rare complications of lymphangioleiomyomatosis (LAM). We report a case of a 50-year-old woman with tuberous sclerosis complex-associated LAM, who presented with expectorating milky-white bronchial casts. She was diagnosed with chyloptysis and chylous pulmonary congestion by sputum analysis. Her symptoms and lung infiltration were improved by oral sirolimus therapy; moreover, serum Krebs von den Lungen-6 (KL-6) levels paralleled the symptoms and lung infiltration of these complications. We suggest that serum KL-6 may be a useful monitoring biomarker of chyloptysis and chylous pulmonary congestion in LAM.

Organizing pneumonia preceding rheumatoid arthritis.

Rheumatoid arthritis patients are susceptible to interstitial lung disease, and joint manifestations of rheumatoid arthritis usually precede lung involvements by several years. Organizing pneumonia, as the first manifestation of rheumatoid arthritis, is extremely rare, and its clinical features remain currently unknown. We present a case and a literature review of patients who were pathologically diagnosed with organizing pneumonia first and met the diagnostic criteria of rheumatoid arthritis later. In this review, we observed the following: (1) patients with organizing pneumonia preceding rheumatoid arthritis have a high prevalence of rheumatoid factor or anticyclic citrullinated peptide antibodies; (2) almost all patients developed rheumatoid arthritis within one year after the diagnosis of organizing pneumonia. We suggest that patients with organizing pneumonia and positive for either rheumatoid factor or anticyclic citrullinated peptide antibody should be cautiously followed up regarding the development of rheumatoid arthritis, particularly during the first year after the diagnosis of organizing pneumonia.

Long-lasting response to crizotinib in brain metastases due to EML4-ALK-rearranged non-small-cell lung cancer.

Anaplastic lymphoma kinase (ALK) rearranged non-small-cell lung cancer (NSCLC) is highly responsive to crizotinib, an oral ATP-competitive selective inhibitor of ALK. However, crizotinib exhibits extremely poor blood-brain barrier penetration; therefore, it is considered to play a limited role in the treatment of brain metastases. We present a case of a 50-year-old man with a diagnosis of ALK-rearranged NSCLC with brain metastasis and malignant pleural effusion. Despite the several systemic chemotherapy regimens and whole brain radiotherapy, brain metastasis was refractory; therefore, crizotinib was initiated. A CT scan showed a slight reduction in the brain metastasis and no change in intrathoracic disease 17 weeks after initiating crizotinib. Moreover, CT obtained 12 months after crizotinib treatment revealed brain metastasis without progression. To our knowledge, the present case is the second report of crizotinib-responsive brain metastases due to echinoderm microtubule-associated protein-like 4-ALK (EML4-ALK)-rearranged NSCLC.

Diffuse alveolar damage associated with pulmonary thromboembolism.

In contrast to other internal organs, pulmonary arterial hypoperfusion does not always show ischemic changes in the lung parenchyma. Pulmonary thromboembolism (PTE)-related lung injury is extremely rare except in the case of pulmonary infarctions, in which PTE occasionally causes necrosis of the parenchyma. We describe the case of an 86-year-old woman who presented with respiratory failure and bilateral ground-glass opacity predominantly the upper lobes. Autopsy revealed a saddle-shaped old organized thrombi in the main pulmonary artery, relatively fresh thrombi in both pulmonary arteries, and localized diffuse alveolar damage (DAD) in the bilateral upper lung fields. The hypoperfused regions resulting from the thromboembolism anatomically coincided with the pulmonary lesion where DAD was identified. Although PTE is not regarded as a causal factor of DAD, it might induce DAD as a result of hypoperfusion in limited cases.