Kouko Hidaka

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.

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.

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.

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.

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.

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 patients 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.

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.

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.

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.