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We herein report a case of presumed septic shock due to Actinotignum schaalii bacteremia with urinary tract infection. A 65-year-old Japanese man suffering from a fever was diagnosed with septic shock due to urinary tract infection. A urine sample was additionally incubated under 5% CO2 and anaerobic conditions after A. schaalii was identified in a blood culture, but A. schaalii was not detected in the urine culture. If Gram-positive rods are observed on Gram staining of a urine sample in symptomatic patients with a predisposing urogenital condition, 5% CO2 and an anaerobic culture of a urine sample should be performed immediately.

Background Yersinia pseudotuberculosis infection can occur in an immunocompromised host. Although rare, bacteremia due to Y. pseudotuberculosis may also occur in immunocompetent hosts. The prognosis and therapeutic strategy, especially for immunocompetent patients with Y. pseudotuberculosis bacteremia, however, remains unknown. Case presentation A 38-year-old Japanese man with a mood disorder presented to our hospital with fever and diarrhea. Chest computed tomography revealed consolidation in the right upper lobe with air bronchograms. He was diagnosed with pneumonia, and treatment with intravenous ceftriaxone and azithromycin was initiated. The ceftriaxone was replaced with doripenem and the azithromycin was discontinued following the detection of Gram-negative rod bacteria in 2 sets of blood culture tests. The isolated Gram-negative rod bacteria were confirmed to be Y. pseudotuberculosis. Thereafter, he developed septic shock. Doripenem was switched to cefmetazole, which was continued for 14 days. He recovered without relapse. Conclusions We herein report a case of septic shock due to Y. pseudotuberculosis infection in an adult immunocompetent patient. The appropriate microorganism tests and antibiotic therapy are necessary to treat patients with Y. pseudotuberculosis bacteremia.

To the Editor,A recent review in the present journal summarized the present knowledge on the virulence of Stenotrophomonas maltophilia [1]. The capability of the agent to act as an opportunistic pa...

Pneumocystis jirovecii pneumonia (PJP) occurs in immunocompromised hosts and is classified as PJP with human immunodeficiency virus (HIV) infection (HIV-PJP) and PJP without HIV infection (non-HIV PJP). Non-HIV PJP rapidly progresses to respiratory failure compared with HIV-PJP possibly due to the difference in immune conditions; namely, the prognosis of non-HIV PJP is worse than that of HIV PJP. However, the diagnosis of non-HIV PJP at the early stage is difficult. Herein, we report a case of severe non-HIV PJP successfully managed with veno-venous extracorporeal membrane oxygenation (V-V ECMO). A 54-year-old woman with neuromyelitis optica was treated with oral corticosteroid, azathioprine, and methotrexate. She admitted to our hospital for fever, dry cough, and dyspnea which developed a week ago. On admission, she required endotracheal intubation and invasive ventilation for hypoxia. A chest computed tomography (CT) scan revealed ground-glass opacity and consolidation in the both lungs. Grocott staining and PCR analysis of bronchoalveolar lavage fluid indicated the presence of fungi and Pneumocystis jirovecii, respectively, whereas serum HIV-antibody was negative. The patient was thus diagnosed with non-HIV PJP and was treated with intravenous pentamidine and corticosteroid pulse therapy for PJP. However, hypoxia was worsened; consequently, V-V ECMO assistance was initiated on day 7. The abnormal chest CT findings and hypoxia were gradually improved. The V-V ECMO support was successfully discontinued on day 14 and mechanical ventilation was discontinued on day 15. V-V ECMO could be a useful choice for respiratory assistance in severe cases of PJP among patients without HIV infection.

Background: Although interferon-γ release assay (IGRA) is widely used for diagnosis of latent tuberculosis (TB) infection, which factors are related to false negative results are uncertain. The aim of this systematic review was to describe the risk factors associated with false negative IGRA results. Methods: Two authors independently identified studies designed to evaluate risk factors for false negative IGRA results from PubMed, the Cochrane Register of Control Trial database, and EMBASE assessed on October 22, 2018. Meta-analyses were conducted with random-effect models and heterogeneity was calculated with the I2 method. Findings: Of 1,377 titles and abstracts screened, 47 full texts were selected for review, and we finally included 17 studies in this systematic review. The most commonly studied risk factor (14 studies) was advanced age followed by low peripheral lymphocyte counts (7 studies), and these factors were associated with false negative results even under different TB prevalence (pooled odds ratio, 2.06; 95%CI, 1.68-2.52 in advanced age, and 7.93; 95%CI, 1.74-36.09 in low peripheral lymphocyte counts, respectively). However, detailed procedure of these assays were not clearly stated in many studies and the cut-off value of these factors were varied. Interpretation: Advanced age and low peripheral lymphocyte counts may be common risk factors for false negative IGRA results, suggesting people with these factors need careful followed-up even if they have negative IGRA results. Standardization of the assay procedure and assessment of risk factors with common definition and the same cut-off value are needed. Funding Statement: The authors state: "There was no funding source for this study." Declaration of Interests: The authors declare declare no competing interests.

Pulmonary lymphoma is rare, accounting for < 1% of primary lung cancers. Most primary pulmonary lymphomas (PPL) are low-grade mucosa-associated lymphoid tissue (MALT)-type, and among PPL, diffuse large B-cell lymphoma (DLBCL) is extremely rare. In contrast, there has been an increase in the incidence of DLBCL among patients with autoimmune disorders and recurrent or chronic bacterial infection. A subset of DLBCL has been reported to develop through transformation of preexisting or concurrent MALT. The respiratory symptoms are non-specific, and the chest X-ray findings demonstrate the presence of interstitial and mixed alveolar infiltrates, nodular lesions, and localized homogeneous consolidations; the diagnosis of pulmonary DLBCL is thus challenging and often leads to a misdiagnosis or delayed diagnosis. We herein report a case of DLBCL which was assumed to have arisen from the lesion of chronic atelectasis that was successfully diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). A 74-year-old woman with diffuse bronchiectasis and chronic atelectasis of the left lower lobe suffered from productive cough and high fever. Increased airway filling with mucoid secretion was repeatedly observed within the area of atelectasis with bronchiectasis, and left lower lobe atelectasis developed. Subsequently, the hilar and mediastinal lymph nodes gradually became enlarged, and DLBCL was pathologically confirmed. In the present case, DLBCL was considered to have arisen in the lesion of chronic atelectasis. Physicians should recognize that DLBCL may develop at the site of chronic atelectasis during disease course of diffuse bronchiectasis, and thus DLBCL may be misdiagnosed as superimposed infection of chronic atelectasis.

Background During veno-venous extracorporeal membrane oxygenation (VV-ECMO), systemic anticoagulation is required to prevent thrombotic complications within the circuit and oxygenator. The unfractionated heparin (UFH) is commonly administered as a standard anticoagulant, but in our institute recombinant human thrombomodulin (rhTM), instead of UFH, is used as an anticoagulant for VV-ECMO. In the present study, we reviewed whether rhTM could be applied effectively and safely as an anticoagulant agent during VV-ECMO. Methods All 15 patients with severe respiratory failure on VV-ECMO were analyzed retrospectively. The following data were collected: age, gender, underlying disease, APACHE-II score, SOFA score, Japanese association for acute medicine (JAAM) DIC score, the usage of anticoagulants, time course of coagulationrelated parameters during ECMO, hemorrhagic and thrombotic complications. Results The median age of the patients was 73 years. The median JAAM DIC score at day 0 was 5 points, indicating that 13 patients were diagnosed with DIC at the initiation of VV-ECMO. The total number of days of VV-ECMO runs combined was 193 days, with a median duration of VV-ECMO of 9 days. Among the 15 VV-ECMO runs, rhTM was used as monotherapy in 5 runs, and a combination of rhTM and (antithrombin) AT was used in 8 runs. UFH was used in combination with rhTM in only 2 runs. Median ACT and aPTT remained a little longer than normal range over the course of the 14 days of a VV-ECMO run. Bleeding events were observed in 6 cases (40%) and no major thromboses were observed in all patients. Conclusions In this retrospective study, we analyzed 15 patients with severe respiratory failure who were administered rhTM as an anticoagulant during VV-ECMO and found that anticoagulation therapy with rhTM is maybe a feasible option which allows for effective and safe VV-ECMO.

Highlights • Actinomyces species colonize the human oropharynx, gastrointestinal tract, and urogenital tract. • Primary sternal osteomyelitis is very rare, especially caused by Actinomyces israelii. • Actinomycosis requires long-term antibiotic treatment. • A. israelii should be included in the differential diagnosis as a causative pathogen of osteomyelitis., We herein report a case of primary sternal osteomyelitis caused by polymicrobial bacteria, including Actinomyces israelii. A 72-year-old man presented with a fever and precordial pain. Chest computed tomography (CT) revealed peristernal fluid associated with an osteolytic lesion and a peripheral nodule in the right upper lobe. We suspected sternal osteomyelitis, and an incision and drainage were performed. Culture of the drainage fluid and bone tissue yielded A. israelii, Fusobacterium necrophorum, and Streptococcus constellatus. Treatment with benzylpenicillin potassium (PCG) was administered. A subsequent chest CT scan showed that the peripheral nodule decreased in size after antimicrobial therapy. We therefore presumed the peripheral nodule as septic pulmonary embolism(SPE). Antimicrobial agents were administered for a total of 6 months. To our knowledge, this is the first case report of primary sternal osteomyelitis associated with presumed SPE caused by polymicrobial bacteria, including A. israelii. It is important to identify the causative pathogen in osteomyelitis, which requires long-term antibiotic treatment.

Tosufloxacin, which is not used to treat Mycobacterium tuberculosis, is a fluoroquinolone recommended for pneumonia when the possibility of tuberculosis infection cannot be excluded. In the present case, symptoms and chest infiltrative shadow initially improved by tosufloxacin. Therefore, we regarded this patient as having general pneumonia and did not perform follow-up chest X-ray until the infiltrates had completely disappeared. However, a few weeks later, the symptoms and the infiltrates had worsened, so M. tuberculosis was isolated from the patient's sputum. This case suggests that patients suspected of having pulmonary tuberculosis should be monitored carefully, even if antibiotics without antituberculous activity are initially effective.

Epithelial cell dysfunction is postulated as an important component in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Mutations in the surfactant protein C (SP-C) gene (SFTPC), an alveolar type II (AT2) cell-restricted protein, have been found in sporadic and familial IPF. To causally link these events, we developed a knockin mouse model capable of regulated expression of an IPF-associated isoleucine-to-threonine substitution at codon 73 (I73T) in Sftpc (SP-CI73T). Tamoxifen-treated SP-CI73T cohorts developed rapid increases in SftpcI73T mRNA and misprocessed proSP-CI73T protein accompanied by increased early mortality (days 7-14). This acute phase was marked by diffuse parenchymal lung injury, tissue infiltration by monocytes, polycellular alveolitis, and elevations in bronchoalveolar lavage and AT2 mRNA content of select inflammatory cytokines. Resolution of alveolitis (2-4 weeks), commensurate with a rise in TGF-β1, was followed by aberrant remodeling marked by collagen deposition, AT2 cell hyperplasia, α-smooth muscle actin-positive (α-SMA-positive) cells, and restrictive lung physiology. The translational relevance of the model was supported by detection of multiple IPF biomarkers previously reported in human cohorts. These data provide proof of principle that mutant SP-C expression in vivo causes spontaneous lung fibrosis, strengthening the role of AT2 cell dysfunction as a key upstream driver of IPF pathogenesis.

Saddle pulmonary embolism (PE) and paradoxical embolism (PDE) are life-threatening disorders carrying a risk of sudden death, and their prompt diagnosis is extremely important. Saddle PE is a radiologic definition and refers to a thrombus that straddles the bifurcation of the pulmonary artery trunk, carrying a risk of sudden hemodynamic collapse. PDE is defined as a systemic arterial embolus due to the passage of a venous thrombus though a right-to-left shunt, such as patent foramen ovale (PFO). We herein present the rare case of asthma exacerbation coincident with saddle PE and PDE. A 69-year-old woman with asthma was suffering from dyspnea, pulse attenuation of the left radial artery and left upper limb pain. An arterial blood gas analysis revealed hypoxemia, and a pulmonary function test demonstrated an obstructive pattern. Enhanced computed tomography (CT) revealed saddle PE, right popliteal venous thrombosis, and left brachial artery occlusion. After the treatment with edoxaban, an anticoagulant, and aspirin, the PE was significantly alleviated, and the brachial artery occlusion was recanalized. Subsequently, the right-to-left shunt through PFO was confirmed, and PDE was suspected of inducting her brachial artery embolism. In the present case, the pulse attenuation of the radial artery and upper limb pain prompted us to consider peripheral vascular disease or coagulation disorders. Physicians should keep in mind that patients with asthma are at considerable risk of PE, and it is important to be aware of possible PFO in patients presenting with the coexistence of PE and systemic arterial embolism.

Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stain-positive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. A 64-year-old woman was admitted to our hospital for blurred vision, muscle weakness of extremities, Raynaud's phenomenon, and exertional dyspnea. We diagnosed her as having systemic sclerosis complicated with sarcoidosis. Chest computed tomography (CT) and transbronchial lung biopsy showed the findings of pulmonary fibrosis without PAP. We treated her with corticosteroid and intravenous cyclophosphamide therapy, followed by tacrolimus therapy. Thereafter, her symptoms improved except for exertional dyspnea, and she began to complain of productive cough thirteen months after corticosteroid and immunosuppressant therapy. On the second admission, a chest CT scan detected the emergence of crazy-paving pattern in bilateral upper lobes. Bronchoalveolar lavage (BAL) fluid with milky appearance and a lung biopsy specimen revealed acellular periodic acid-schiff stain-positive bodies. The serum titer of anti-granulocyte macrophage colony stimulating factor (GM-CSF) antibodies was elevated on first admission and remained high on second admission. We thus diagnosed her as having autoimmune PAP. Reducing the dose of immunosuppressive agents and repeating the segmental BAL resulted in the improvement of her symptoms and radiological findings. Immunosuppressant therapy may trigger the onset of autoimmune PAP in a subset of patients with systemic sclerosis and/or sarcoidosis.

A 64-year-old man was prescribed maoto, a prevailing Chinese herbal, for a cold with upper respiratory inflammation. Two days later, he developed a high fever, progressive dyspnea and pulmonary infiltration on chest high-resolution computed tomography (HRCT) including diffuse ground-glass opacity mainly around bronchovascular bundles and partial distribution of subpleural cysts resembling honeycombing. Despite the administration of azithromycin and pazufloxacin, the pulmonary infiltration and hypoxemia has rapidly progressed, so he was referred to our hospital. Although fulminant pneumonia or the acute exacerbation of idiopathic pulmonary fibrosis (IPF) was considered, his respiratory symptoms and pulmonary infiltration immediately improved and oxygen therapy was not needed on the fifth hospital day. Based on the clinical course, laboratory findings and the chest imaging findings, drug induced interstitial lung disease was suspected. The drug-induced lymphocyte test (DLST) as well as a scratch test against maoto demonstrated positive results. This is the first case report of maoto-induced interstitial pneumonia that was diagnosed based on the patient’s clinical course, chest imaging findings and laboratory findings.

Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by the infiltration of IgG4-bearing plasma cells into systemic organs. Although 25% to 35% of patients with RP have a concurrent autoimmune disease, coexistence of RP and IgG4-RD is rare. We herein report a case of RP complicated by IgG4-RD. A 63-year-old man developed recurrent bilateral ear pain and swelling, recurrent blurred and decreased vision, and migratory multiple joint pain, sequentially within one year. Fourteen months after the first symptom, he experienced dry cough and dyspnea with exertion. A computed tomography (CT) scan detected interstitial pneumonia, swelling of bilateral submandibular glands, bilateral hilar and mediastinal lymphadenopathy, and several nodules in bilateral kidneys. His serum levels of IgG and IgG4 were elevated. The biopsy specimen of auricular cartilage showed infiltrations of inflammatory cells and fibrosis consistent with RP. The IgG4-positive cells were not observed in auricular cartilage. The patient met the diagnostic criteria of RP, including bilateral auricular chondritis, conjunctivitis, iritis and polyarthritis. The biopsy specimens of lung and kidney revealed the significant infiltrations of IgG4-positive plasma cells and fibrosis. We also diagnosed him as having IgG4-RD, affecting bilateral submandibular glands, hilar and mediastinal lymph nodes, lungs, and kidneys. Thus, RP preceded the onset of IgG4-RD. Corticosteroid therapy improved the symptoms and CT scan findings. In conclusion, RP and IgG4-RD do coexist; however, the pathogenesis of their coexistence is unknown.

A 42-year-old woman presented with an intermittent fever and chest and back pain, and an abnormal chest shadow was detected. She was diagnosed with paragonimiasis caused by Paragonimus westermani. Praziquantel therapy improved the abnormal chest shadow, but did not relieve her symptoms. She was also diagnosed with familial Mediterranean fever (FMF), and colchicine therapy resolved her symptoms. She subsequently developed arthralgia and morning stiffness in her hands. We also diagnosed the patient with rheumatoid arthritis (RA), and corticosteroid and salazosulfapyridine therapy improved her symptoms. The existence of paragonimiasis complicated the diagnosis of FMF. The coexistence of FMF and RA is very rare, but does exist.

Fluorodeoxyglucose (FDG)-positron emission tomography with computed tomography (FDG-PET/CT) is useful in disease monitoring of malignancies after therapy, while an FDG uptake may also be present in benign diseases. We herein demonstrate a case of disseminated Mycobacterium tuberculosis mimicking systemic metastasis of prostate cancer. This case highlights that clinicians should consider Mycobacterium tuberculosis in patients with prostate cancer who demonstrate multifocal FDG uptakes masquerading as metastasis, even when the chest photographs reveal a normal appearance and a sputum examination demonstrates negative results. An invasive surgical biopsy may be required and a pathological analysis would be critical in the diagnosis of Mycobacterium tuberculosis.

Here, we report a case of pneumonia possibly caused by the aspiration of runoff water containing Chromobacterium haemolyticum. A 69-year-old man became intoxicated with alcohol, fell into a ditch, and lost consciousness after suffering a blow to the head. The lower half of his body was completely paralyzed because of damage to his spinal cord, and he aspirated runoff water from the ditch. Chest computed tomography scans revealed consolidation in the right upper lobe and bilateral lower lobes. A sputum culture detected gram-negative bacteria that was identified as C. haemolyticum. Antibacterial chemotherapy was initiated, and the clinical course was favorable. To the best of our knowledge, this is the first report of C. haemolyticum pneumonia in the literature.

Summary Background Pulmonary fibrosis is a manifestation of microscopic polyangitis (MPA), and often precedes the detection of MPA. The prevalence and sequence of myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) and MPA in patients initially diagnosed with idiopathic pulmonary fibrosis (IPF) have not been precisely elucidated. Methods We enrolled 61 consecutive patients with IPF and measured the MPO-ANCA titers at initial presentation and during the follow-up period. Clinical, radiologic and histologic features of MPO-ANCA-positive cases were examined. Results Of 61 patients, 3 (4.9%) had positive MPO-ANCA titers at the initial presentation of IPF. During the disease course, MPO-ANCA-positive conversion occurred in 6 patients and the prevalence of ANCA increased to 14.8%. Among the nine patients positive for MPO-ANCA, two patients developed MPA during follow-up. Histologic features of MPO-ANCA-positive pulmonary fibrosis were compatible with the usual interstitial pneumonia pattern in which alveolar hemorrhage and capillaritis were not observed. The patients with MPO-ANCA-positive conversion showed increased percentages of bronchoalveolar lavage eosinophils and more frequent complication of pulmonary emphysema compared to those with MPO-ANCA-negative IPF. Conclusions The findings of the present study demonstrated that patients with an initial diagnosis of IPF occasionally acquire MPO-ANCA, which develops to MPA during the disease course of IPF. The presence of pulmonary eosinophilia and low attenuation areas on computed tomography scans might be predictive of MPO-ANCA positive conversion.

Epithelial cell dysfunction and abnormal wound healing have reemerged as central concepts in the pathophysiology of a variety of parenchymal diseases of the distal adult lung including idiopathic pulmonary fibrosis, asbestosis, Hermansky–Pudlak-related fibrosis, and chronic obstructive pulmonary disease in adults as well as interstitial lung disease in children. Alveolar type 2 (AT2) cells represent a metabolically active compartment of the distal lung responsible for pulmonary surfactant biosynthesis and are emerging as an important progenitor population required for maintenance of alveolar homeostasis. This chapter leverages reviews keys aspects of AT2 cell and surfactant biology relevant to the pathogenesis of chronic lung disease. Leveraging mutations in surfactant system components as model substrates, the aberrant responses to the intrinsic challenge of mutant protein conformers as well as exogenous environmental insults (e.g., tobacco smoke) including endoplasmic reticulum stress, inflammatory/profibrotic signaling, mitochondrial dysfunction, and intrinsic apoptosis are discussed. The role of cell quality control (QC) pathways that can be utilized to defend AT2 homeostasis and their derangement in disease will be placed in the context of cell stress and QC signatures found in patients with parenchymal lung remodeling along with their implications for personalized therapeutic strategies for chronic parenchymal lung diseases based on cellular endophenotypes.

Fluorodeoxyglucose (FDG)-positron emission tomography with computed tomography (FDG-PET/CT) is useful in disease monitoring of malignancies after therapy, while an FDG uptake may also be present in benign diseases. We herein demonstrate a case of disseminated Mycobacterium tuberculosis mimicking systemic metastasis of prostate cancer. This case highlights that clinicians should consider Mycobacterium tuberculosis in patients with prostate cancer who demonstrate multifocal FDG uptakes masquerading as metastasis, even when the chest photographs reveal a normal appearance and a sputum examination demonstrates negative results. An invasive surgical biopsy may be required and a pathological analysis would be critical in the diagnosis of Mycobacterium tuberculosis.

Epithelial cell dysfunction has emerged as an important factor in Idiopathic Pulmonary Fibrosis (IPF) pathogenesis. Mutations in the Surfactant Protein C [SP-C] gene [SFTPC], an alveolar type 2 cell [AT2] restricted protein, have been linked to sporadic and familial forms of diffuse parenchymal lung disease [DPLD] including IPF. The …

We herein report a rare case of sarcoidosis presenting as bilateral vocal cord paralysis due to bilateral vagal nerve involvement. A 72-year-old woman with uveitis of the left eye complained of hoarseness and aspiration due to bilateral vocal cord paralysis. An endobronchial needle aspiration biopsy specimen of the mediastinal lymph nodes showed non-caseating epithelioid cell granuloma. Total protein and cell concentrations in the cerebrospinal fluid were increased. We diagnosed her to have sarcoidosis with bilateral vagal nerve involvement. Corticosteroid therapy improved her symptoms of hoarseness and aspiration. Sarcoidosis should therefore be taken into consideration as a potential cause of bilateral vocal cord paralysis.

Primary malignant melanoma of the trachea is extremely rare. We report here the first case of primary tracheal malignant melanoma in the asymptomatic stage. Incidentally, this 73-year-old man was found to have a flat tumor at the upper trachea on chest computed tomography scans. The tumor was surgically resected with end-to-end anastomosis and was diagnosed to be primary malignant melanoma of the trachea. Four months after the surgical resection, cervical lymph node metastasis was found. Despite the resection of metastatic lymph nodes and six courses of chemotherapy, he died of cachexia approximately two years after the discovery of the tracheal tumor.

Summary A 36-year-old man was transferred to the hospital for further evaluation of pulmonary infiltration. A diagnosis of acute eosinophilic pneumonia (AEP) was confirmed by clinical symptoms, bronchoalveolar lavage, and computed tomography findings. Skin tests with fungal antigens were performed by intradermal injection. Both the Arthus (8h) and delay (24h)-type skin tests were positive for only Candida albicans . A lymphocyte-stimulating test was also positive for C. albicans . The etiology of the AEP was confirmed by a C. albicans inhalation provocation test. In addition, peripheral blood mononuclear cells obtained from the patient produced Interleukin-5 following C. albicans stimulation. This is the first report of C. albicans as a probable cause of AEP. Evaluation of allergy to C. albicans should be performed in AEP before diagnosing the cause as idiopathic.

Subcutaneous sarcoidosis is the specific subset of cutaneous sarcoidosis frequently associated with systemic disease. However, the disease activity, severity, and prognosis have not yet been elucidated due to the limited number of reported cases. The purpose of this study was to identify the clinical, laboratory, and prognostic differences between subcutaneous sarcoidosis and other type of cutaneous sarcoidosis. All patients with sarcoidosis diagnosed histopathologically from 2000 to 2012 at our institution were enrolled. The clinical, laboratory, chest X-ray, and pulmonary function test results were retrospectively evaluated in the patients with cutaneous sarcoidosis. In the 130 patients with sarcoidosis, cutaneous sarcoidosis was diagnosed in 37 patients (28.4 %), and 9 (6.9 %) of these patients had subcutaneous sarcoidosis. The serum levels of soluble interleukin-2 receptor (sIL-2R) were significantly elevated in the group of patients with subcutaneous sarcoidosis in comparison to the patients with other types of cutaneous sarcoidosis, whereas there was no significant difference in the severity score between the two groups. Following a 2-year observation period, three patients were in remission, five patients demonstrated stable disease, and one patient had progressive disease. Subcutaneous sarcoidosis may be associated with the disease activity, although it was not found to be associated with the disease severity and it was not a predictive factor for the prognosis. Furthermore, the prevalence of subcutaneous sarcoidosis may be higher than that in previously reported series.

Dating back nearly 35 years ago to the Witschi hypothesis, epithelial cell dysfunction and abnormal wound healing have reemerged as central concepts in the pathophysiology of idiopathic pulmonary fibrosis (IPF) in adults and in interstitial lung disease in children. Alveolar type 2 (AT2) cells represent a metabolically active compartment in the distal air spaces responsible for pulmonary surfactant biosynthesis and function as a progenitor population required for maintenance of alveolar integrity. Rare mutations in surfactant system components have provided new clues to understanding broader questions regarding the role of AT2 cell dysfunction in the pathophysiology of fibrotic lung diseases. Drawing on data generated from a variety of model systems expressing disease-related surfactant component mutations [surfactant proteins A and C (SP-A and SP-C); the lipid transporter ABCA3], this review will examine the concept of epithelial dysfunction in fibrotic lung disease, provide an update on AT2 cell and surfactant biology, summarize cellular responses to mutant surfactant components [including endoplasmic reticulum (ER) stress, mitochondrial dysfunction, and intrinsic apoptosis], and examine quality control pathways (unfolded protein response, the ubiquitin-proteasome system, macroautophagy) that can be utilized to restore AT2 homeostasis. This integrated response and its derangement will be placed in the context of cell stress and quality control signatures found in patients with familial or sporadic IPF as well as non-surfactant-related AT2 cell dysfunction syndromes associated with a fibrotic lung phenotype. Finally, the need for targeted therapeutic strategies for pulmonary fibrosis that address epithelial ER stress, its downstream signaling, and cell quality control are discussed.

Here, we report a case of pneumonia possibly caused by the aspiration of runoff water containing Chromobacterium haemolyticum. A 69-year-old man became intoxicated with alcohol, fell into a ditch, and lost consciousness after suffering a blow to the head. The lower half of his body was completely paralyzed because of damage to his spinal cord, and he aspirated runoff water from the ditch. Chest computed tomography scans revealed consolidation in the right upper lobe and bilateral lower lobes. A sputum culture detected gram-negative bacteria that was identified as C. haemolyticum. Antibacterial chemotherapy was initiated, and the clinical course was favorable. To the best of our knowledge, this is the first report of C. haemolyticum pneumonia in the literature.

Background: ADAM (a disintegrin and metalloprotease) family members, characterized by a metalloprotease and a disintegrin domain, are membrane-anchored glycoproteins involved in proteolysis and cell adhesion. ADAM8 might have an important role in allergic inflammation. It can cleave a variety of substrates and is a sheddase for VCAM-1 and CD23, the low-affinity IgE receptors. Methods: To evaluate the contribution of ADAM8 to the pathogenesis of eosinophilic pneumonia (EP), we measured the concentrations of soluble ADAM8 (sADAM8) and its substrates, soluble VCAM-1 (sVCAM-1) and soluble CD23 (sCD23), in bronchoalveolar lavage fluid from patients with smoking-induced acute eosinophilic pneumonia (AEP), chronic idiopathic eosinophilic pneumonia (CEP), and drug-induced eosinophilic pneumonia (drug-EP). Results: The sADAM8 and sVCAM-1 concentrations were increased in AEP and CEP. The sCD23 concentration was elevated in AEP. In AEP, but not CEP, the sADAM8 concentration significantly correlated with those of both sVCAM and sCD23. Conclusion: The pathogenesis of AEP, CEP, and drug-EP was distinct with regard to ADAM8. Our results are the first to associate ADAM8 with eosinophilic responses and lung inflammation in humans.

A disintegrin and metalloprotease (ADAM) family members, characterized by a metalloprotease and a disintegrin domain, are membrane-anchored glycoproteins involved in proteolysis and cell adhesion. ADAM8 is specifically induced in the experimental murine asthmatic lung. To evaluate novel pathways involved in asthma pathogenesis, using ADAM8 transgenic mice (ATMS2) in a murine model of asthma. Massive cellular infiltrates in peribronchovascular and interstitial lesions were observed in control mice, while in ATMS2 mice there were only occasional. Vascular cell adhesion molecule (VCAM-1) is involved in specific eosinophil adhesions via alpha4beta1 integrin. VCAM-1 shedding was mediated by the ADAM8 metalloprotease. Endothelial cell shedding of VCAM-1 was increased in ATMS2-stimulated human umbilical endothelial cells. ADAM8-mediated shedding of VCAM-1 might be important for the suppression of experimental asthma. Our data suggest that ADAM8 is a useful therapeutic target.