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Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that causes abnormal blood vessel formation and bleeding. We herein report a 61-year-old woman with aggravated HHT symptoms after hemodialysis initiation. She was diagnosed with HHT based on her recurrent bleeding, abnormal blood vessel formation, and family history. Despite bleeding complications, the patient required anticoagulants and antiplatelet agents to treat cardiovascular complications. Eventually, the patient died of extensive cerebral hemorrhaging. Our experience suggests that special attention should be paid to bleeding complications in high-risk patients.

Allergic bronchopulmonary aspergillosis (ABPA) and chronic pulmonary aspergillosis (CPA) are diseases caused by Aspergillus infection, and CPA can develop from ABPA in some cases. We herein report a patient with CPA overlapping with ABPA. Serum cytokine levels were evaluated at 4 time points: the ABPA diagnosis, CPA diagnosis, 6 months after the start of voriconazole (VRCZ), and 12 months after re-administration of VRCZ. Interleukin (IL)-13 levels decreased upon glucocorticoid treatment, whereas IL-25 and IL-33 levels decreased rapidly with the initiation of antifungals. Early antifungal therapy may be important to control disease progression and prevent CPA overlap.

A 78-year-old male was admitted to the hospital with acute renal failure and generalized erythema after starting dapagliflozin 10 mg/day for chronic kidney disease (CKD). A skin biopsy revealed superficial perivascular dermatitis with eosinophils. A renal biopsy revealed lymphocytic and eosinophilic infiltration of the interstitium, and focal tubulitis. The patient was diagnosed with a dapagliflozin-induced drug reaction with eosinophilia and systemic symptoms (DRESS), followed by acute interstitial nephritis (AIN), and prednisolone therapy was therefore initiated. The patient's renal function improved, and erythema disappeared. To our knowledge, this is the first report of DRESS caused by dapagliflozin, and the patient was successfully treated with prednisolone.

Tuberculous meningitis is an infectious disease with high mortality. Literature describing intrathecal therapy for tuberculous meningitis is scarce. We herein report a case of refractory tuberculous meningitis in a 52-year-old woman with underlying neuropsychiatric systemic lupus erythematosus. Despite systemic treatment with anti-tuberculosis drugs and dexamethasone, her meningeal irritation deteriorated. Intrathecal isoniazid and prednisolone administration was therefore initiated, and the symptoms of severe meningeal irritation improved along with head magnetic resonance imaging and cerebrospinal fluid findings. This case report highlights the efficacy of intrathecal isoniazid and steroid injections for refractory tuberculous meningitis, particularly in patients with severe meningeal irritation.

A 77-year-old Japanese woman with mediastinal lymphadenopathy and uveitis was diagnosed with sarcoidosis. The bacterial flora in biopsied samples from mediastinal lymph nodes was analyzed using a clone library method with Sanger sequencing of the 16S rRNA gene, and Streptococcus gordonii (52 of 71 clones) and Cutibacterium acnes (19 of 71 clones) were detected. No previous study has conducted a bacterial floral analysis using the Sanger method for the mediastinal lymph node in sarcoidosis, making this case report the first to document the presence of S. gordonii and C. acnes in the mediastinal lymph node of a patient with sarcoidosis.

A 36-year-old Japanese man presented with cavities and nodular shadows in the lower lobes of his lungs and osteolytic lesions in the thoracic spine. He was diagnosed with multisystem Langerhans cell histiocytosis (LCH). Three years earlier, he had been noted to have small cavities and granular lesions noted in the upper lobes of his lungs, which later improved with smoking cessation. It was likely that his single-system pulmonary LCH (PLCH) progressed to multisystem LCH despite smoking cessation. Relapse or progression may occur in cases where PLCH lesions improve after smoking cessation. Thus, close follow-up is vital.

Pulmonary nodular lymphoid hyperplasia (PNLH) is a very rare disease, and it is difficult to diagnose PNLH and distinguish it from mucosa-associated lymphoid tissue (MALT) lymphoma. In addition, information on bronchoalveolar lavage fluid (BALF) analyses is lacking. We herein report a 36-year-old Japanese woman diagnosed with PLNH by a surgical biopsy and analysis of BALF. The BALF showed an increase in B-cell marker-positive lymphocytes, normal patterns of B-cell clonality, mucosa-associated lymphoid tissue 1 gene, and immunoglobulin heavy chain at 14q32 translocations. We also reviewed Japanese cases of PNLH described in Japanese or English to explore the characteristics of such cases.

A 26-year-old Japanese woman was admitted with a 1-month history of diarrhea, a high fever for a few days, and exacerbation of dyspnea. She was treated with an antifibrotic drug and long-term oxygen therapy for Hermansky-Pudlak syndrome-related pulmonary fibrosis. New ground-glass attenuation appeared on chest computed tomography (CT), and a colon biopsy showed an inflammatory cell accumulation with a high titer of myeloperoxidase (MPO)-specific anti-neutrophil cytoplasmic antibodies (ANCA). Systemic inflammation related to MPO-ANCA titer elevation was suspected. Steroid pulse therapy and intravenous cyclophosphamide improved chest CT findings and diarrhea. Therefore, immunosuppressant treatment should be considered for systemic inflammation related to MPO-ANCA.

A 65-year-old Japanese man with interstitial pneumonia demonstrated honeycomb lung with thickened walls on chest high-resolution computed tomography (HRCT) and predominance of neutrophils in the cell fraction of the bronchoalveolar lavage fluid. Although there were no centrilobular nodular or branching shadows on chest HRCT suggestive of diffuse panbronchiolitis, he exhibited sinusitis and had the human leukocyte antigen (HLA)-B54 antigen. With long-term macrolide therapy, the cough and sputum production markedly improved, wall thickening of the honeycomb lung on chest HRCT decreased, and the forced vital capacity increased. Confirming the presence of HLA-B54 antigen may help determine the indication for long-term macrolide therapy in interstitial pneumonia patients.

Objective The quality of life and activities of daily living (ADL) are generally poor among dialysis patients after intracerebral hemorrhaging, and their precise clinical course remains unclear. In addition, the association between the severity of cerebral hemorrhaging and the long-term prognosis in these patients has not been fully elucidated. This study aimed to evaluate the subsequent prognosis of hemodialysis patients who survived the acute phase of intracerebral hemorrhaging. Methods We included hemodialysis patients who were admitted to Nagasaki University Hospital between 2007 and 2015 for intracerebral hemorrhaging treatment. After excluding cases of in-hospital death, survivors were classified using the 5-point modified Rankin Scale (mRS), which specifically measures the ADL in patients with cerebrovascular diseases. The patients were followed up at the medical facilities to which they were transferred in the same medical zone until 2017. Results Out of 91 patients with cerebral hemorrhaging (65±11 years old, 66% men, hemodialysis duration 108±91 months), 62 survived until discharge. Twenty-one patients died during observation, largely due to infectious diseases, such as sepsis and pneumonia (n=16, 76%). Compared to patients with mRS 0-4 (n=31), those with mRS 5 (n=31) showed a significantly poorer prognosis. The hazard ratio adjusted for age and antiplatelets was 13.7 (95% confidence interval: 3.88-63.7, p<0.001). Conclusion Hemodialysis patients with intracerebral hemorrhaging who were bedridden showed poor outcomes. The major causes of death were infections. Therefore, these patients should be carefully monitored for infections in order to improve their prognosis.

A 68-year-old woman developed systemic blisters while receiving treatment for nephrotic syndrome. As she also developed marked liver dysfunction and disseminated intravascular coagulation, she was admitted to our hospital. She was diagnosed with varicella zoster virus (VZV) infection. Treatment was administered in the intensive-care unit, but the patient died on day 24 post-admission after severe VZV infection. A post-mortem examination showed micro-abscesses and necrosis caused by varicella zoster infection in multiple organs, including the liver, kidneys, and gastrointestinal tract. Because VZV infection can become severe in immunocompromised patients, careful consideration is needed for the prevention and treatment of the viral infection.

A 45-year-old Japanese man was referred to our hospital with chest and back pain with a duration of two days. Contrast-enhanced computed tomography (CT) showed a large calcified lesion in the inferior vena cava (IVC), thrombus with calcification in the pulmonary arteries of the left lower lobe, and an infiltrative shadow in the left lower lobe. He was diagnosed with pulmonary thromboembolism (PE) and pneumonia. Calcification in the IVC was not evident on any CT images obtained six and eight years earlier. This patient was identified to be an extremely rare case of PE associated with IVC calcification that developed during adulthood.

Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody is associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM). We herein report three Japanese cases of anti-MDA5 antibody-positive RP-ILD without signs of CADM or DM. High-resolution computed tomography revealed patchy or subpleural distribution of consolidations and/or ground-glass opacities accompanied by traction bronchiectasis. All patients succumbed to respiratory failure within two months. Anti-MDA5 antibody-positive RP-ILD without signs of CADM or DM should be included in the differential diagnosis of acute/subacute ILD. Measurement of anti-MDA5 antibody and an intensive immunosuppressive regimen might rescue these patients from RP-ILD.

A 44-year-old man who received allogenic hematopoietic stem cell transplantation after being diagnosed with acute myeloid leukemia developed nephrosis when the dose of tacrolimus was tapered. A renal biopsy showed the granular deposition of immunoglobulin G in the glomerular basement membrane and subepithelial electron-dense deposits, crescent formation, C4d-positive staining of the peritubular capillary, and subendothelial swelling, suggesting that the main pathological diagnosis was membranous nephropathy and that chronic graft-versus-host disease played a role in the etiology of nephrosis. We herein report a case of membranous nephropathy with various pathological findings. C4d deposition suggests complement activation and the involvement of humoral factors.

Scedosporium apiospermum is an opportunistic fungus that can cause various types of infections, including localized infections and life-threatening disseminated infections, particularly in immunocompromised patients. Treatment is especially challenging due to its multidrug resistance. We herein report the case of a 73-year-old woman who was non-immunocompromised but developed S. apiospermum lung infection and a pulmonary tumorlet. To our knowledge, this is the first report of the coexistence of pulmonary S. apiospermum infection and tumorlet. The lung lesion was successfully treated by surgical excision without any antifungal agents, and no recurrence of the tumorlet or S. apiospermum infection has occurred.

A 65-year-old Japanese man was referred to our hospital for the further assessment of cough and dyspnea. He had a history of ulcerative colitis for which he was receiving treatment. Chest computed tomography showed a crazy-paving pattern. His bronchoalveolar lavage fluid had a milky appearance, and a transbronchial lung biopsy specimen revealed acellular periodic acid-Schiff stain-positive bodies. The serum anti-granulocyte macrophage-colony stimulating factor (GM-CSF) antibody titer was elevated. The diagnosis was autoimmune pulmonary alveolar proteinosis (PAP). There are few reports of autoimmune PAP in patients with ulcerative colitis. Some reports suggest that PAP and inflammatory bowel disease might have a common pathogenesis involving the anti-GM-CSF antibody.

We herein report a rare case of oral mucormycosis following allogeneic hematopoietic stem cell transplantation. Oral mucormycosis due to Rhizopus microsporus manifested as localized left buccal mucositis with a 1-cm black focus before neutrophil recovery. Combination therapy with liposomal amphotericin B was initiated and surgical debridement was performed; however, the patient died due to progressive generalized mucormycosis. Considerable attention needs to be paid to the diagnosis and management of oral mucormycosis in post-transplant patients, thereby suggesting the importance of fully understanding the risk factors.

Although pneumothorax has been reported to be a major pulmonary adverse event in patients treated with pazopanib, a multikinase inhibitor, drug-induced interstitial lung disease (DILD) has not been reported. A 74-year-old Japanese man who received pazopanib for the treatment of femoral leiomyosarcoma and lung metastasis presented with dyspnea and fatigue. He had mild interstitial pneumonia when pazopanib treatment was initiated. Chest computed tomography revealed progressive bilateral ground-glass opacity (GGO) and traction bronchiectasis. We diagnosed DILD due to pazopanib. The patient's pazopanib treatment was interrupted and a steroid was administered. The symptoms and GGO were improved with treatment. Physicians should be aware of DILD due to pazopanib in patients with pre-existing interstitial lung disease.

Xeroderma pigmentosum (XP) is a genetic disease in which DNA repair mechanisms are impaired. Cisplatin (CDDP) exerts cytotoxic effects by forming mainly intrastrand DNA cross-links, and sensitivity to CDDP depends on the DNA repair system. Several in vitro studies have suggested that treatment with CDDP may cause enhanced adverse events as well as anti-tumor activity in cancer patients with XP. This article is the first to describe two cancer patients with XP showing severe adverse events following CDDP-based chemotherapy. Physicians should pay attention when administering CDDP in cancer patients with XP.

Pulmonary actinomycosis is a rare pulmonary infection that often exhibits unspecific symptoms and radiological findings. We herein report a case of pulmonary actinomycosis that mimicked pulmonary aspergilloma in an immunocompetent patient.

Pulmonary artery sarcoma is highly malignant and easily metastasizes to the systemic organs. Both the introduction of novel diagnostic procedures and the development of new treatment modalities are required to achieve long-term survival. Several studies have shown that platelet-derived growth factor receptor α (PDGFRα) gene amplification is frequently observed in pulmonary artery sarcoma. PDGFRα is known to be involved in cell proliferation in certain malignancies. PDGFRα may become a potential biological marker in pulmonary artery sarcoma. We report a case in which a diagnosis of pulmonary artery sarcoma overexpressing PDGFRα was made using endovascular catheter biopsy following positron emission tomography with integrated computed tomography (PET/CT) scans.

A 35-year-old non-HIV patient developed pulmonary cryptococcosis after the initiation of infliximab. He recovered by fluconazole treatment and completed the therapy for a total of 6 months. However, he experienced a relapse 16 months later during retreatment with infliximab, revealing an interesting clinical course contradicting retreatment. This case also represents the first case of relapsed pulmonary cryptococcosis suspected during treatment with a biologic agent. Both of these aspects generated important clinical questions about the length of pulmonary cryptococcosis treatment and the necessity of introducing a second prophylaxis for such patients.

A 37-year-old woman was admitted to a hospital due to a prolonged fever and a rash on her legs. She had systemic lymphadenitis and a skin abscess on her left leg. Pathological findings of a left leg skin biopsy revealed abscess formation with granulomatous dermatitis, Mycobacterium abscessus complex was cultured from the resected left supraclavicular lymph node, and disseminated M. abscessus complex infection was diagnosed. She was treated with combination treatment with antimicrobials and percutaneous drainage, and her clinical findings improved. Four months later, she developed acute lymphocytic leukemia. Leukemia is a risk factor for disseminated M. abscessus complex infection, even before developing leukemia.

Tracheobronchopathia osteochondroplastica (TO) is a rare benign airway disease that is characterized by the presence of multiple rock-garden-like nodules on bronchoscopy. TO is a slowly progressive disease of the trachea and major bronchi, which is typically characterized by such symptoms as a persistent nonproductive cough, dyspnea and wheezing. The clinical features of TO are variable, and asymptomatic patients may incidentally be diagnosed during the work-up for other diseases. We herein report a rare case of TO accompanying multiple tracheobronchial polyposis in which bronchoscopic resection of the airway polyp using a high-frequency snare was successfully performed.

A 23-year-old Japanese woman was admitted to A hospital due to pneumonia. IgM for Mycoplasma pneumoniae was positive, and the patient was treated with imipenem/cilastatin, clindamycin, pazufloxacin and minocycline. However, both the chest radiological findings and the symptoms became exacerbated, and she was therefore transferred to our hospital. The bronchoalveolar lavage fluid was obtained, and a 16S rRNA gene sequencing analysis revealed a monobacterial infection of Mycoplasma pneumoniae. Therefore, corticosteroid treatment in addition to minocycline was administered, and the patients symptoms, laboratory data and chest radiographs improved. Corticosteroid therapy may therefore be considered for patients with refractory M. pneumoniae pneumonia.

Streptococcal toxic shock syndrome caused by group B streptococcus (GBS) is a rare, but lethal disease. We experienced a 45-year-old woman with pustular psoriasis who developed toxic shock-like syndrome during infliximab treatment. Surprisingly, similar episodes recurred three times in one year with restarting of infliximab treatments. In the third episode, GBS were detected in blood, urine, and vaginal secretion cultures. These episodes of shock syndrome were possibly due to GBS. To the best of our knowledge, this is the first case report of recurrent streptococcal toxic shock syndrome possibly caused by GBS which was induced by anti-TNF-α inhibitor therapy. The restarting of biological agents in patients with a history of toxic shock syndrome should therefore be avoided as much as possible.

We herein report a case of IgG4-related lung disease (IgG4-RLD) associated with autoimmune hemolytic anemia (AIHA). A 73-year-old Japanese female visited our hospital for an examination following an abnormal chest X-ray in 1999. She was diagnosed with bronchiolitis and AIHA, and treatment with prednisolone was started. After seven years, she visited our department due to a cough. Chest computed tomography (CT) demonstrated focal consolidation with ground-glass attenuations and thickened bronchial walls in the bilateral lungs. She was clinically diagnosed and treated for bronchial asthma. CT findings had shown no changes, and a lung biopsy was performed using video-assisted thoracic surgery at eleven years from the first diagnosis of AIHA. The pathological findings demonstrated the presence of peribronchovascular lymphoplasmacytic infiltrates with stromal fibrotic changes, admixed with many IgG4-positive plasma cells. Furthermore, the patient's serum IgG4 level was high, and her CT findings did not show any obvious abnormal findings in the any organs other than the lungs. She was diagnosed with IgG4-RLD based on the findings. We believe that this case report of IgG4-RLD associated with AIHA is clinically helpful for a better understanding of these diseases, although there are five reported cases of IgG4-related disease associated with AIHA.

Primary mediastinal large B-cell lymphoma (PMLBCL) is one of the subtypes of diffuse large B-cell lymphoma. We experienced a rare case of PMLBCL that exhibited endobronchial involvement. A 33-year-old Japanese female with the chief complaints of epigastralgia, back pain, and nausea visited a primary care hospital. Computed tomography of the chest and abdomen demonstrated a bulky mass in the left anterior mediastinum, multiple pulmonary nodules, axillary lymph node swelling, and a pancreatic tumor. Fiberoptic bronchoscopy showed a white-tinged irregularly shaped endobronchial tumor accompanied by capillary vessel dilation in the left upper lobar bronchus. Taken together, these findings resulted in a diagnosis of PMLBCL.

A 75-year-old man with interstitial pneumonia and enlarged mediastinal lymph nodes underwent endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). He developed a high-grade fever seven days after EBUS-TBNA was performed; laboratory and radiologic findings showed intense inflammatory reactions, with swelling of the mediastinal lymph nodes on chest computed tomography. Mediastinal lymph node abscess was diagnosed, and it worsened in spite of systemic antibacterial treatment. Surgical treatment using a median sternotomy was performed, and the cultivation of surgically obtained mediastinal lymph node abscess fluid revealed Streptococcus intermedius. Combined treatment with antibiotics and surgical treatment was effective, leading to remission.

A 75-year-old Japanese man underwent thoracic surgery to treat a large lung cancer lesion in the left upper lobe with calcified pleural thickening. Postoperatively, viable Mycobacterium tuberculosis was detected in the margin of the resected thickened calcified pleural lesion. Therefore, an infection control investigation of medical staff who had come in contact with the patient was conducted. Consequently, two of the 14 healthcare professionals who had been in the operating room were diagnosed with latent tuberculous infections. Therefore, strict precautions against airborne infections are required to prevent the in-hospital transmission of M. tuberculosis in such cases.

Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder, the most common complication of which influencing the prognosis is pulmonary fibrosis. In the present report, we describe an autopsy case of a Japanese woman with HPS. The patient was diagnosed at 50 years of age based on the presence of oculocutaneous albinism, hemorrhagic diathesis, ceroid-lipofuscin accumulation and pulmonary fibrosis. Although systemic steroids, immunosuppressants and pirfenidone were administered for pulmonary involvement, she died from respiratory failure two years later. Obtaining an early diagnosis and taking into consideration the need for lung transplantation is necessary in order to improve the prognosis of HPS. We herein report this very rare Japanese case of HPS with a review of the treatment approaches for HPS complicated with pulmonary fibrosis.

A 47-year-old man was referred to our hospital with an abnormal shadow on a chest X-ray. He had a history of untreated chronic sinusitis and suspected ulcerative colitis (UC). Chest CT revealed a diffuse centrilobular granular shadow, while laboratory tests demonstrated an increased proportion of neutrophils; however, no microorganisms were detected in bronchoalveolar lavage fluid. Therefore, sinobronchial syndrome or small airway disease associated with UC was diagnosed, and the patient was treated with long-term erythromycin therapy. Small airway disease associated with UC is usually treated with steroids. Our experience shows that airway involvement in patients with inflammatory bowel disease can be treated with macrolides.

The bacterial species in the Streptococcus anginosus group (S. constellatus, S. anginosus, S. intermedius) are important causative pathogens of bacterial pneumonia, pulmonary abscesses and empyema. However, the bacteria in this group are primarily oral resident bacteria and unable to grow significantly on ordinary aerobic culture media. We experienced a case of pneumonia and empyema caused by Streptococcus intermedius detected using a 16S rRNA gene sequencing analysis of bronchoalveolar lavage fluid and pleural effusion, but not sputum. Even when applying the molecular method, sputum samples are occasionally unsuitable for identifying the causative pathogens of lower respiratory tract infections.

We herein report a rare case of pulmonary mucosa-associated lymphoid tissue (p-MALT) lymphoma with multiple cystic lesions. A previously healthy 58-year-old Japanese woman visited our hospital for an evaluation of abnormal chest computed tomography (CT) findings. Chest CT revealed multiple cystic lesions in both lungs, and she was diagnosed as having p-MALT lymphoma based on the pathological findings. The patient had no underlying autoimmune diseases. She has not received any chemotherapy and has been stable for two years. This case suggests that, although rare, the possibility of p-MALT lymphoma should be considered in patients with multiple cystic lung diseases.

We herein present a case of Good syndrome complicated by diffuse pulmonary lesions similar to diffuse panbronchiolitis (DPB). A 45-year-old Japanese man was referred to our department due to recurrent lower respiratory tract infections that had started and ameliorated nine months after thymectomy for pure red cell aplasia and myasthenia gravis. Diffuse centrilobular opacities on chest computed tomography and positivity for HLA-B54 were consistent with DPB. Additionally, hypogammaglobulinemia and a marked decrease of B-lymphocytes were observed, and therefore Good syndrome was considered. Combination therapy with azithromycin and clarithromycin alleviated the patient's respiratory symptoms and reduced the exacerbation of chronic bronchitis.

Objective: The prognosis of idiopathic pulmonary fibrosis (IPF) patients with acute exacerbation (AE) is reported to be extremely poor. Several clinical studies suggest that direct hemoperfusion with polymyxin B-immobilized fiber (PMX) may have beneficial effects on AE in patients with interstitial pneumonia (IP). The aim of this multicenter retrospective analysis was to investigate whether PMX treatment could provide improvement of oxygenation and survival benefits in IPF patients with AE., Methods: We conducted a retrospective study of 160 IP patients (including 73 IPF) with AE treated by PMX at 18 institutions in Japan. PMX treatment was carried out twice. The total hemoperfusion time of PMX treatment was, on average, 12 hours. Data concerning oxygenation on PMX treatment and survival after AE were collected and analyzed., Results: In IPF patients with AE, arterial oxygen tension (PaO(2))/inspiratory oxygen fraction (FiO(2)), (P/F) ratio was significantly improved at the end of the 2nd treatment with PMX (173.9 ± 105.4 to 195.2 ± 106.8 Torr, p=0.003). White blood cell count was significantly reduced at the end of the 2nd treatment (13,330 ± 7,002 to 9,426 ± 5,188 /mm(3), p<0.001). These clinical changes were also observed on analysis of all 160 IP patients with AE. The one- and three-month survival rates of IPF patients after AE were 70.1% and 34.4%, respectively., Conclusion: PMX treatment may improve oxygenation and survival in IPF patients with AE. Prospective, controlled trials of PMX treatment for IPF with AE are warranted to verify this potential benefit.

We herein report two cases of primary ciliary dyskinesia (PCD) with different responses to macrolides. Case 1: a 17-year-old Japanese man with Pseudomonas aeruginosa infection and combined defect of both inner and outer dynein arms in the cilia was unsuccessfully treated with long-term macrolides (clarithromycin, erythromycin, and azithromycin). Case 2: a 70-year-old Japanese man with deficiency of only the inner dynein arm was successfully treated with clarithromycin. Though the reasons for the different responses to macrolides are unclear, differences of ultrastructural abnormalities of the cilia might be one of the predictive factors in PCD just as in Pseudomonas aeruginosa infection.

We herein report two cases of everolimus-associated interstitial pneumonia in patients with renal cell carcinoma. A 68-year-old Japanese man (case 1) was admitted to our hospital because of progressive dyspnea, left infiltration and consolidation on chest radiographs. He had started receiving everolimus (10 mg daily) three months before the admission for the treatment of recurrent renal cell carcinoma. Bronchoalveolar lavage fluid taken from his left B(4) showed a marked increase of lymphocytes (42.9%). An organizing pneumonia pattern of everolimus-associated interstitial pneumonia was strongly suspected radiologically, and treatment with high-dose corticosteroids, discontinuation of everolimus and oxygen support was started. The treatment was successful, and the patient recovered with only minor pulmonary fibrotic changes in the left lower lobe. A 57-year-old Japanese man (case 2) was referred to our department for the evaluation of interstitial pneumonia. He had started to receive everolimus (10 mg daily) four months previously. Chest CT demonstrated interstitial pneumonia predominantly in bilateral lower lobes, with small pulmonary metastatic nodules. His pulmonary complications were spontaneously resolved eight days after the discontinuation of everolimus. To the best of our knowledge, Case 1 is the first reported case of successfully treated organizing pneumonia pattern of interstitial pneumonia with acute respiratory failure induced by everolimus in Japan.

We report a case of acute respiratory distress syndrome (ARDS) after influenza A infection who was successfully treated with combined treatment including direct hemoperfusion with polymyxin B-immobilized fiber (PMX-DHP) column. A 56-year-old Japanese man was admitted to our hospital in January 2010 because of progressive dyspnea, hypoxemia, fever and bilateral diffuse infiltration on chest radiograph after pandemic influenza A infection. His chest computed tomography showed diffuse and patchy bilateral ground-glass opacities, and we diagnosed ARDS after influenza A infection. The patient was successfully treated with PMX-DHP in addition to the treatment with oseltamivir, corticosteroid, sivelestat and antibiotics with mechanical ventilation, and the patient recovered with only minor pulmonary fibrotic change. Although the efficacy of PMX-DHP treatment in patients with acute lung injury (ALI)/ARDS after influenza virus infection is not well established, this treatment could be a possible therapeutic modality in treating the patients with this disease.

A 73-year-old woman who had been diagnosed with systemic sclerosis was admitted for further examination of bilateral hilar lymphadenopathy. Sarcoidosis was confirmed based on elevated serum levels of angiotensin-converting enzyme, a high proportion of lymphocytes and a high CD4/CD8 ratio in bronchoalveolar lavage fluid, abnormal (67)Gallium uptake in the mediastinum and noncaseating granulomas in skin biopsy specimens. In addition, high levels of antimitochondrial M2 antibodies and alkaline phosphatase indicated primary biliary cirrhosis (PBC). Here we describe a rare triplex of sarcoidosis, SSc and PBC. Although the etiology of this complex remains unknown, these three diseases might share some pathogenesis.

Diffuse alveolar hemorrhage (DAH) syndrome is potentially fatal. We encountered a nearly fatal case of DAH possibly due to intravenous itraconazole (ITCZ). A 53-year-old man with chronic pulmonary aspergillosis underwent pneumonectomy of the left lung 15 days prior to the onset of DAH, which was confirmed by bronchoalveloar lavage. The battery of diagnostic evaluations performed revealed no other positive etiological factor, leading to the diagnosis of DAH possibly induced by intravenous ITCZ with a positive drug lymphocyte stimulation test. The patient did not respond to pulse methylprednisolone therapy, but responded dramatically to direct hemoperfusion using a polymyxin B-immobilized fiber column (PMX) therapy.

A 51-year-old man was admitted to our hospital with cough, hemosputum, dyspnea and chest pain. Chest high-resolution computed tomography (HRCT) revealed diffuse ground-glass opacities in both lungs with peripheral predominance. Bronchoalveolar lavage fluid was fresh-bloody and analysis indicated an increase in the eosinophil proportion. Benzbromarone-induced lymphocyte stimulation test was positive. Therefore, the patient was diagnosed as having drug-induced eosinophilic pneumonia with pulmonary alveolar hemorrhage caused by benzbromarone. After discontinuation of benzbromarone and administration of corticosteroids, chest HRCT images and respiratory manifestation improved. Here, we report this rare case of benzbromarone-induced eosinophilic pneumonia with pulmonary alveolar hemorrhage.

A 46-year-old man was referred to our hospital with hemoptysis. He had been diagnosed with chronic sinusitis since childhood, but had received no treatment. Chest CT showed a diffuse centrilobular granular shadow and thickened bronchial walls. Otitis media and decreased spermatic motor ability were identified. In addition, electron microscopy of a biopsy specimen of the nasal mucosa showed a deficiency of inner dynein. Based on these clinical findings, primary ciliary dyskinesia (PCD) was diagnosed and successfully treated with long-term, low-dose clarithromycin. Although the effects of macrolide therapy remain controversial, long-term treatment with low-dose clarithromycin might confer clinical benefits upon patients with PCD.

Objective: Idiopathic interstitial pneumonias (IIPs) comprises a group of diffuse parenchymal lung diseases of unknown etiology with varying degrees of inflammation and fibrosis including cryptogenic organizing pneumonia (COP), idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP). Tenascin-C is an extracellular matrix molecule that is expressed during wound healing in various tissues. The present study was aimed to investigate the role of tenascin-C in the pathogenesis of IIPs., Methods: We used enzyme-linked immunosorbent assays to measure levels of tenascin-C in serum and bronchoalveolar lavage fluid (BALF) from 17 patients with IPF, 12 with NSIP, 15 with COP and from 23 healthy individuals., Results: Serum levels of tenascin-C were significantly elevated in patients with COP compared with those in all other participants, whereas those in patients with IPF and NSIP were not significantly elevated compared with healthy individuals. The levels of tenascin-C in BALF from patients with COP and NSIP were significantly higher than those of healthy individuals. In addition, serum tenascin-C was significantly correlated with levels of serum C-reactive protein, which is a serum acute phase protein., Conclusion: Systemic inflammation in the lung with IIPs might be associated with tenascin-C. These results suggest that tenascin-C is responsible for the pathogenesis of IIPs especially via inflammation, and that it might serve as a serum marker of COP.

Cystic fibrosis (CF), the most common lethal hereditary disorder in Caucasians, is quite rare in Southeast Asia including Japan. Here, we report three CF cases encountered in Nagasaki, Japan. Case 1; a 24-year-old man with dyspnea and cough was diagnosed as CF with a missense mutation Q98R in exon 4 and a polymorphic 125C in exon 1 in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Case 2; a 13-year-old woman born of consanguineous parents was diagnosed as CF with homozygous Q98R mutations in exon 4. Case 3; a 29-year-old woman complaining of cough and sputum was diagnosed as CF with a heterozygous R347H mutation in exon 7 and a polymorphic 125C in exon 1. These mutations have been previously reported in Caucasian patients, but are considered very rare. Although the numbers of individuals with CF are very limited, the profiles of CFTR mutations in those patients are likely diverse in Japan.

Desquamative interstitial pneumonia (DIP) is a rare pattern of diffuse parenchymal lung disease known as one of the idiopathic interstitial pneumonias and is considered to be a smoking- or dust inhalation-related interstitial pneumonia in the majority of cases. This report presents the first case of DIP in which the pulmonary manifestation preceded the onset of rheumatoid arthritis. This case and our review of twenty-four DIP cases (nineteen cases previously-reported from Japan, plus five cases in our departments) indicate the possibility that the DIP pattern is an additional form of diffuse interstitial pneumonia that may develop in association with autoimmune diseases.

A 74-year-old man with poorly controlled diabetes mellitus was admitted to our hospital because of severe respiratory disturbance, fever, and sputum. We found massive consolidation of the right lung and nodular shadows on the left lung on chest X-ray, and detected influenza virus and Streptococcus pneumoniae antigen from a nasopharyngeal swab and urine sample, respectively. Co-infection with influenza virus and bacteria was suspected, and oseltamivir and biapenem were prescribed. Laboratory data improved after the addition of sivelestat sodium hydrate, an inhibitor of neutrophil-derived elastase; however, chest X-ray findings became worse on Day 8, and we administered 1 g methylprednisolone intravenously for two days. On Day 12, we detected Mycobacterium tuberculosis in the sputum, even though we did not previously detect any acid-fast bacilli, and started anti-tuberculosis drugs, such as isoniazid, rifampicin, ethambutol hydrochloride, and pyrazinamide; however, the patient died 12 days later. Severe influenza-related bacterial pneumonia with Streptococcus pneumoniae and subsequently secondary tuberculosis infection were finally suspected in this case. This was a very rare case in which additional tuberculosis infection was found in a patient with fulminant pneumonia due to co-infection of influenza virus and bacteria. It is necessary to observe patients with influenza carefully, especially when steroids are used, even if antibiotics are also administered.