Successful intervention for overwhelming postsplenectomy infection caused by non-vaccine pneumococcal serotype 23A.

The spleen plays an important role in the body's immune defense against invasive infections, particularly those caused by encapsulated bacteria. Encapsulated bacterial infection in asplenic patients is a medical emergency called overwhelming postsplenectomy infection (OPSI) and has a mortality rate of 50-70%. Here, we report the case of a 51-year-old Asian man who complained of emesis and diarrhea as primary symptoms. He rapidly progressed to coma and was eventually diagnosed with OPSI (pyogenic ventriculitis/spondylitis) caused by non-vaccine pneumococcal serotype 23A. Aggressive management, including empiric antibiotic therapy, a staircase approach for intracranial pressure-targeted therapy and laminectomy/laminoplasty, resulted in a good recovery. Our report highlights that non-vaccine pneumococcal serotypes can cause disease in vaccinated patients.

Basal cell adenoma of the lung identified with obstructive pneumonia.

The seromucinous glands of the bronchi can give rise to tumors resembling those of the salivary glands. Basal cell adenoma (BCA) has not been reported in salivary gland-type tumors of the lung. Here, we report a case of an 86-year-old Asian man who suffered from pneumonia in the left upper lung field that may be associated with an endobronchial tumor occluding B1 + 2a + b. The bronchoscopic biopsy of the tumor revealed that the basaloid cells, which constituted a major component of the tumor and were positive for p40/p63 immunohistochemistry, exhibited a cord-like structure. The Ki-67 (MKI67) staining was less than 1% positive. These findings suggest that the endobronchial tumor was BCA of the lung, which caused obstructive pneumonia in the patient.

Life-threatening pyogenic ventriculitis, complicating endocarditis, presenting as sudden hearing loss.

Approximately 70% of sudden sensorineural hearing loss (SSNHL) cases are idiopathic, but SSNHL may occasionally be related to fatality. Here, we report the case of a 54-year-old Asian female who complained of hearing loss as a primary symptom and was diagnosed with pyogenic ventriculitis (PV) derived from Streptococcus agalactiae endocarditis. Both PV and S. agalactiae endocarditis are rare and very severe infections with a high mortality rate. Rapid intensive treatment and surgery were required for successful clinical outcomes.

Poorly differentiated adenocarcinoma of an unknown primary with a thyroid tumour and an aggressive course: thyroid or lung carcinoma?

Cancers of unknown primary (CUPs) are challenging for physicians to diagnose and treat. Metastases to the thyroid gland are rare, representing less than 1% of all thyroid malignancies. Here, we report a case of a 69-year-old Asian man who had both thyroid gland and lymph node enlargement in the neck and shoulders but no nodules/tumours in the lung field. The patient died 51 days after his first visit to our office, although pembrolizumab was administered on day 34 based on programmed cell death-ligand 1 (PD-L1) expression. Immunohistochemistry (IHC) with paired box 8 (PAX8) may be useful to diagnostically distinguish poorly differentiated lung adenocarcinomas from napsin A-positive thyroid carcinomas.

Insufficient initial treatment but good recovery after diagnosis of pulmonary actinomycosis.

Actinomycosis is an infrequent invasive bacterial disease. Although multiple different clinical features of actinomycosis have been described, pulmonary actinomycosis (PA) is a rare but challenging diagnosis and complete cure to make. Here we report the case of a 41-year-old Asian woman who had a mass-like consolidation in the right middle lung field. The lung lesion was exacerbated after initial treatment. After diagnosis of PA, thoracoscopic resection combined with intravenous and oral penicillin made a good recovery.

Delayed diagnosis of odontogenic infection: a lesson from altered mental status and abnormal breathing.

Altered mental status is a common, yet challenging, clinical presentation encountered by physicians. Here, we report a case of a 68-year-old Japanese female who was transferred to the emergency department due to faint. The laboratory results showed hyponatremia, ketonuria, hyperglycemia and acute kidney injury without fever or inflammatory findings. Although these abnormalities were corrected, her mental status was exacerbated, and apnea/tachypnea appeared. She was eventually diagnosed with acute apical abscess and recovered immediately after dental extractions. This case suggests that odontogenic infection should be considered in the differential diagnosis of altered mental status and that interdisciplinary dental management, including surgical treatment, should be considered for patients with predisposing factors.

[A case of cytokeratin 19 positive extramammary Paget's disease with pulmonary metastases].

An 82-year-old woman was admitted because of progressing cough and dyspnea. Two years prior to admission, she had undergone an operation for extramammary Paget's disease which resected the primary lesion, the left labium majora, and ipsilateral inguinal and obturator lymph nodes. Thoracic images showed diffuse infiltrative shadows and bilateral effusions. Repeated thoracenteses revealed no microbes or malignant cells. Antibiotic treatment was ineffective. Transbronchial lung biopsy specimens revealed Paget cells, and immunohistochemical stains confirmed pulmonary metastases of Paget's disease. Cytokeratin 19 fragments were positive in the serum, pleural effusion, and lung biopsy specimens. The patient died of respiratory failure 3 years after the initial diagnosis of extramammary Paget's disease.

Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis.

STUDY OBJECTIVES: To evaluate the long-term clinical course of patients with idiopathic pulmonary fibrosis (IPF) complicated with pulmonary arterial hypertension. DESIGN: Prospective analysis of consecutive IPF patients undergoing initial workup with right-heart catheterization (RHC) and pulmonary function testing (PFT). Pulmonary arterial pressure (PAP) and diffusion capacity of the lung for carbon monoxide (Dlco) were focused on. SETTING: University hospital. PATIENTS: Seventy-eight patients with IPF (67 men, 11 women; diagnosis by pathology, n = 59; clinical diagnosis, n = 19) had been followed up after initial workup for a maximum of 14 years. MEASUREMENTS AND RESULTS: RHC data on 61 patients and PFT data on 52 patients were available. Five-year survival rates were 62.2% in the normal-PAP group (mean PAP < 17 mm Hg, n = 37) and 16.7% in the high-PAP group (mean PAP > 17 mm Hg, n = 24) [p < 0.001]; 70.4% in the preserved-Dlco group (percentage of predicted > 40%, n = 27) and 20.0% in the low-Dlco group (percentage of predicted < 40%, n = 25) [p < 0.001]; and 82.6% in group 1 (normal PAP and preserved Dlco, n = 23) and 15.6% in group 2 (high PAP, low Dlco, or both, n = 32) [p < 0.0001]. The relative risks of mortality within 5 years after RHC were 2.20 (95% confidence interval [CI], 1.40 to 3.45) in the high-PAP group, 2.70 (95% CI, 1.46 to 4.99) in the low-Dlco group, and 4.85 (95% CI, 1.97 to 11.97) in group 2. CONCLUSION: Dlco was a critical factor for evaluating disease status and prognosis, and PAP status provided feasible information in the initial workup of IPF patients.

[A case of idiopathic nonspecific interstitial pneumonia and autoimmune hemolytic anemia after complete cure of polymyalgia rheumatica].

In 1997, at the age of 68, a man was admitted with polymyalgia rheumatica, which was successfully treated with oral prednisolone. In 1999, a chest X-ray revealed that he had interstitial changes in both lung fields. Because there were no symptoms, he was observed without treatment. However, from around 2003, he began to experience gradual progression of severe dyspnea on exertion. He was admitted to our hospital in 2004, at the age of 75, and we found interstitial lung deterioration. Nonspecific interstitial pneumonia (NSIP) was diagnosed because of the increase in the number of lymphocytes in bronchoalveolar lavage fluid and CT findings. He was also found to have autoimmune hemolytic anemia (AIHA). Treatment of AIHA with 1mg kg prednisolone led to improvement of not only the anemia but also the interstitial pneumonia. The coexistence of both idiopathic interstitial pneumonia and autoimmune hemolytic anemia is very rare. Moreover, this is the first report of polymyalgia rheumatica occurring prior to these two diseases.

Pulmonary infection of Mycobacterium avium-intracellulare complex with simultaneous organizing pneumonia.

A 67-year-old woman presented high-grade fever and dyspnea. Sputum culture confirmed Mycobacterium avium-intracellulare complex (MAC). Transbronchial lung biopsies revealed organizing pneumonia (OP) that was rapidly improved with corticosteroid. Five months after onset, a nodule emerged in the right lung. Although MAC was confirmed, the lesion was deemed too small to merit anti-mycobacterial chemotherapy. Four months later, diffuse infiltrates developed on chest X-ray. Bronchoalveolar lavage study identified MAC and exhibited OP patterns. We commenced antimycobacterial chemotherapy. The infiltrates almost completely improved within a month without corticosteroid.

Cell profiles of bronchoalveolar lavage fluid as prognosticators of idiopathic pulmonary fibrosis/usual interstitial pneumonia among Japanese Patients.

BACKGROUND: The role of bronchoalveolar lavage fluid (BALF) cell profiles in predicting the clinical outcome of idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP) is still under discussion. OBJECTIVE: To determine whether BALF cell profiles affect the survival of patients with UIP diagnosed by surgical lung biopsy/autopsy at the early stage of IPF. METHODS: This hospital-based retrospective cohort study used 81 Japanese patients with histologically proven IPF/UIP who underwent BAL examination. The BALF samples were obtained from non-current smokers: NCS (n = 41) and current smokers: CS (n = 40). The Kaplan-Meier and Cox's proportional hazard methods were used to estimate the survival and evaluate the risk ratio for death in the two groups. To detect the multicollinearity, a stepwise regression was employed. RESULTS: A slight increase in the absolute numbers of BALF neutrophils tended to relate to a decrease in the relative risk for death in NCS patients and CS patients in the univariate analysis. In stepwise regression, the increase in percent vital capacity and the increase in the BALF CD4/CD8 ratio in NCS was detected as a favorable predictor, while increased BALF cells affected the results due to chronic smoking in CS. CONCLUSIONS: Based on the study bias of the biopsy-proven IPF/UIP patients at stable stages, an independent variable indicating a favorable outcome was an increased BALF CD4/CD8 ratio in NCS patients, while it was difficult to identify definite prognosticators in CS patients.

Patient characteristics and clinical features of Japanese sarcoidosis patients with low bronchoalveolar lavage CD4/CD8 ratios.

BACKGROUND: Previous studies have shown that there is an association between low BALF (bronchoalveolar lavage fluid) CD4/CD8 ratio and lower remission rates in sarcoidosis patients. AIM: To investigate the patient characteristics and clinical features of Japanese sarcoidosis patients with low BALF CD4/CD8 ratios. METHODS: 322 Japanese sarcoidosis patients were retrospectively analyzed, and 3 groups were selected according to BALF CD4/CD8 ratios as follows: patients with the BALF CD4/CD8 ratio in the lowest 5 percentile (Group 1: 0.43-1.41), median 5 percentile (Group II: 4.68-5.47), and top 5 percentile (Group III: 12.6-60.1). Each group consisted of 16 patients (5% of 322 patients). The patient characteristics, clinical features, and the short-term prognosis for at least 2 years (average 116 months) were compared among the groups. Multivariate analysis was performed for 322 patients to investigate the determinants of BALF CD4/CD8 ratios. RESULTS: The number of BALF CD8+ cells were greater in Group I than in the other two groups. In Group I, there were higher incidences of younger age, male gender, and lower number of extrathoracic lesions compared with Group III. Multivariate analysis showed that younger age and male gender were independently associated with low BALF CD4/CD8 ratios. The frequency of treatment with corticosteroid and progression to pulmonary fibrosis tended to be higher in Group I. CONCLUSIONS: Low BALF CD4/CD8 ratios were due to increased number of BALF CD8+ cells. Younger age and male gender were independently associated with low BALF CD4/CD8 ratios in sarcoidosis patients.

Polymorphisms of B7 (CD80 and CD86) genes do not affect disease susceptibility to sarcoidosis.

BACKGROUND: B7 proteins (CD80, CD86) are costimulatory molecules expressed on antigen-presenting cells and are essential factors for T cell activation. OBJECTIVES: The aim of this study is to investigate the relationship of B7 gene polymorphisms either to disease susceptibility or to cell profile of bronchoalveolar lavage (BAL) fluid in Japanese sarcoidosis patients. METHODS: Gene polymorphisms located in CD80 promoter, CD80 exon 3 and exon 8 of CD86 were examined in 146 Japanese sarcoidosis patients and 157 healthy controls using single-strand conformation polymorphism and direct sequencing. The distribution of genotypes was compared between the two groups. BAL fluid cell profiles were compared for the various genotypes of the different polymorphisms for the 62 patients who underwent BAL. RESULTS: There were no significant differences in the distribution of genotypes or allele frequencies for all polymorphisms between sarcoidosis and controls. There were no significant differences in BAL fluid cell profiles among the different genotype groups of the various polymorphisms studied. CONCLUSIONS: There was no relationship between the B7 gene polymorphisms studied and disease susceptibility or BAL fluid cell profiles in Japanese sarcoidosis patients.

Multiple bone fractures found in a young sarcoidosis patient with long stable disease.

A 22-year-old Japanese man was found to have bilateral hilar lymphadenopathy (BHL), and was diagnosed with sarcoidosis in 1995. He was followed without treatment until 2002, when a bone fracture due to osseous sarcoidosis was found in his left thumb. Despite systemic treatment with corticosteroid and methotrexate, a new bone lesion developed in his right foot and his right middle finger was fractured. The patient also suffered multiple organ involvements including brain and muscle lesions. This is the first report of a sarcoidosis patient who presented with BHL, and developed bone fractures after a long stable period of more than 5 years.

Diabetes insipidus from neurosarcoidosis: long-term follow-up for more than eight years.

Four patients with sarcoidosis presented as hypothalamic-hypophyseal syndrome including diabetes insipidus (DI) were followed up for more than 8 years from the onset of clinical manifestation. The mean age was 26 years, male : female ratio was 3 : 1 and the mean disease duration of 10 years. All patients had hypogonadism, hyperprolactinemia. Pituitary enlargement with thickening of the pituitary stalk were detected by magnetic resonance imaging (MRI) with gadolinium enhancement and attenuation in the intensity of the posterior lobe of the pituitary was detected without enhancement. Corticosteroid therapy resulted in the initial improvement of symptoms and gradual decrease in the tumor size but failed to cure polyuria due to DI. The use of desmopressin was necessary for a long period. None of these patients died from DI or central neurosarcoidosis.

Cytotoxic T-lymphocyte antigen-4 (CTLA-4) exon 1 polymorphism affects lymphocyte profiles in bronchoalveolar lavage of patients with sarcoidosis.

BACKGROUND: Sarcoidosis is a systemic disease characterized by T-cell activation and subsequent granuloma formation at the site of involvement. Genetic susceptibility is a key factor in the pathogenesis of this disease, and genes involved in T-cell regulation are potential candidates. Cytotoxic T-lymphocyte antigen-4 (CTLA-4) is a negative regulator of T-cell activation expressed on activated T-cells. The G allele of the CTLA-4 exon 1 polymorphism has previously been described to be associated with disease susceptibility in several autoimmune diseases. We investigated the relationship of CTLA-4 to disease susceptibility and cell profiles in bronchoalveolar lavage (BAL) in Japanese sarcoidosis patients. METHODS: Japanese sarcoidosis patients (n = 135) and controls (n = 97) were typed for an A/G bi-allelic polymorphism in exon 1 of CTLA-4 using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP), and the distribution of genotypes was compared between both groups. Sixty-seven patients underwent BAL, and cell profiles in BAL fluid were compared between patients with G/G genotype and those with A/A genotype. RESULTS: No significant differences in the distribution of genotype and allele frequencies were found between sarcoidosis (GG: 46%, AG: 39%, AA: 15%) and controls (GG: 42%, AG: 49%, AA: 8%). Patients with G/G genotype had significantly increased lymphocyte ratios, lymphocyte counts, and CD4(+) cell counts in BAL fluid compared with patients with A/A genotype (p < 0.05). CONCLUSIONS: CTLA-4 exonl polymorphism might affect BAL fluid lymphocyte profiles in Japanese sarcoidosis patients.

Sarcoidosis complicated with IgA nephropathy.

Though IgA nephropathy (IgAN) and sarcoidosis have been reported simultaneously in patients occasionally, it remains controversial whether there is any association between the two. In this report we describe our experience with one definite and two subclinical cases of IgAN diagnosed during the clinical course of sarcoidosis. Our findings suggest a possible association between these disorders, and a higher incidence of subclinical IgAN among sarcoidosis patients than among the general population. We also discuss the common immunological background shared by these two diseases.

Cyclophosphamide-induced late-onset lung disease.

We report the case of a 58-year-old woman who was considered to have died of cyclophosphamide (CPA)-induced late-onset lung disease. She underwent a right-sided mastectomy due to breast cancer at the age of 50 followed by daily administration of 50 mg CPA and 20 mg tamoxifen for 2 years. A refractory cough and dyspnea began at the age of 56. Chest radiographs revealed diffuse infiltrates and pleural thickening. Her vital capacity was markedly reduced. Corticosteroid therapy was ineffective. The disease rapidly progressed with occasional episodes of pneumothoraces to her death. Postmortem examination revealed pulmonary fibrosis with marked elastosis.

Sarcoidosis with bilateral epididymal and testicular lesions.

We report a case of genital sarcoidosis that presented characteristic features on MRI. A 25-year-old man sarcoidosis patient with ocular and lung lesions presented a painful mass in the left scrotum together with systemic symptoms of fever, appetite loss, headache, and stomach-ache during the tapering of steroids. The patient was hypercalcemic, and this was thought to be the cause of his systemic symptoms. MRI showed multiple nodules of bilateral testes and enlargement of bilateral epididymis; the patient was diagnosed with testicular and epididymal lesions of sarcoidosis. An increased steroid dosage improved his hypercalcemia and genital lesions.