[Idiopathic pleuroparenehymal fibroelastosis: five case reports and review of literature].

Objective: To raise the awareness of idiopathic pleuroparenehymal fibroelastosis (iPPFE) through investigating the clinical, radiographic and pathological features. Methods: Five cases of iPPFE proved by pathology. The clinical data were studied respectively, and the relevant literature was reviewed. Results: All the cases of iPPFE were manifested by cough and dyspnea. The patients including 3 males and 2 females, aged from 30 to 70 years Chest CT scan showed pleural thickening, subpleural consolidation in both upper lungs complicated with tractive bronchiectasis.Computed tomography-guided percutaneous lung biopsy or surgical lung were performed and the same pathological showed pleura and subpleural dense elastic and collagen fibers. The elastic fibers stain was also positive,which was consistent with PPFE. One patient received low-dose corticosteroid, two received pirfenidone therapy, the others received no treatment. Three patients were stable during the follow-up. Conclusions: iPPFE has characteristic pathological features. However, the number of clinically reported cases is low due to missed diagnosis or misdiagnosed. Improving the understanding of features of iPPFE is helpful for the dianosis, therapy, and prognosis of this disease.

[The clinical significance of myositis-specific antibodies in polymyositis/dermatomyositis associated interstitial lung diseases].

Objective: To investigate the profile and clinical significance of myositis-specific antibody spectrum (MSAs) in patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Methods: Sera from 74 patients with PM/DM-ILD, 29 patients with SLE and 32 healthy controls were collected and Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs . The clinical data of all patients were collected from medical records. Statistical analysis was performed using One-way ANOVA, t-test, rank sum test, χ(2) test or Fisher's exact test. Results: The overall detection rate of MSAs in 74 patients with PM/DM-ILD was 86.5%, significantly higher than that in patients with SLE and healthy controls (χ(2)=66.24, 69.85, P<0.01). According to the diagnostic criteria of PM/DM, 18 of 74 patients were definitely diagnosed, 11 were preliminarily diagnosed and 45 were suspected, in which the detection rate of MSA was 83.3%, 90.9% and 86.7%, respectively .The detection rates of MSAs in 17 PM-ILD and 57 DM-ILD were 82.4% and 87.7% respectively. The anti-ARS and anti-MDA5 were the two most common subtypes of MSAs in patients with PM/DM-ILD, the positive rates being 59.5% and 25.7%, respectively . The incidence of CADM, acute/subacute ILD and 90-day mortality in the anti-MDA5 positive group (χ(2)=12.945, 23.203, 26.434, P<0.05) was significantly higher than those of the anti-ARS group and the MSA-negative group, while the incidence of helitrope rash, V-rash, fever was significantly higher than the anti-ARS positive group (χ(2)=11.462, 5.895, 10.609, P<0.05). The incidence of muscle weakness in anti-Jo-1 group was significantly higher than that in the non-Jo-1 antibody group (χ(2)=3.991, P<0.05), while other clinical features were not statistically significant between the anti-Jo-1 and the non-Jo-1 anti-ARS positive groups (P>0.05). Conclusion: The detection rate and accuracy of MSAs in polymyositis/dermatomyositis with ILD was very high, which was useful for early diagnosis of the disease, and severity and prognosis assessment. It is strongly recommended that MSAs should be detected in patients with clinical suspicion of PM/DM-associated interstitial lung diseases.

[Unilateral pleural effusion as first manifestation in Takayasu arteritis: a case report and review of literature].

Objective: To highlight the characteristics of pulmonary arterial involvement in Takayasu arteritis. Methods: The clinical and radiological data of a patient with Takayasu arteritis presenting with unilateral pleural effusion were studied and relevant literature was reviewed. The key words, "Takayasu arteritis" and "pleural effusion" were analyzed through literature retrieval in databases. Results: This 58 year-old female patient presented with shortness of breath. The chest CT scan showed bilateral hilar enlargement and pleural effusion on the left side. The blood pressure was not measurable in the course of the disease. After the aorticopulmonary-arteriography, we found that the pulmonary artery and the subclavian artery were involved. The diagnosis of Takayasu arteritis was made, and glucocorticoid therapy was initiated, with significant clinical and radiological improvement after therapy. Literature review found 4 cases of Takayasu arteritis with unilateral pleura effusion, ranging from 32 to 35 years of age, with a female predominance(Female∶Male=3∶1). The chief complaints were fever, chest pain and hemoptysis. All the patients recovered after the treatment of glucocorticoids. Conclusions: Takayasu arteritis presenting with unilateral pleural effusion was easily misdiagnosed as primary pulmonary diseases. Careful physical examination and timely angiography can be used to make the diagnosis.

[Comparison of naming impairment among patients with Parkinson's disease without dementia, Parkinson's disease dementia and Alzheimer's disease].

OBJECTIVE: To compare the characteristics of naming impairment in Parkinson's disease (PD) without dementia, Parkinson's disease dementia (PDD) and Alzheimer's disease (AD). METHODS: All participants came from outpatient clinic or inpatients of our hospital during 2011-2013. Object and action naming tests were evaluated among patients with PD without dementia (n=60), PDD (n=60), AD (n=60) and healthy control group (n=60). RESULTS: The object naming score of PD without dementia group was 40.2±2.8, PDD group was 36.0±3.1, AD group was 31.6±4.0 and healthy control group was 44.1±2.2, while the action naming score of PD without dementia group was 27.3±2.6, PDD group was 20.5±4.0, AD group was 22.5±2.7 and healthy control group was 31.6±1.4. The object and action naming were both impaired in PD without dementia, PDD and AD patients compared with healthy control group (P<0.01). CONCLUSIONS: PD patients without dementia have slight object and action naming impairments with more impairments in action naming. Action naming is more impaired in PDD patients, while object naming is more impaired in AD patients.

Tuberculoma masked by glioma: a case report.

Tuberculous meningitis (TM), a common infectious disease of the central nervous system that is also seen in other types of tuberculosis infections, has higher mortality rates in young and middle-aged patients. TM is difficult to diagnose and treat owing to its non-specific clinical features and often atypical cerebrospinal fluid changes. Patients who present with focal neurologic signs, cough, low-grade fever and illness duration of more than 5 days, have intracalvarial abnormalities, and do not meet Thwaites' criterion findings should be diagnosed using computed tomography or magnetic resonance imaging. Mycobacterium infections can also be diagnosed by acid-fast staining of smears, cerebrospinal fluid culture, diagnostic polymerase chain reaction for Mycobacterium tuberculosis, and purified protein derivative test. To prevent TM misdiagnosis, clinicians must have sufficient knowledge of the clinical manifestations of tuberculosis. Appropriate application of tuberculosis chemotherapy drug principles, including early diagnosis and treatment, combination therapies, and consistent administration of treatment at appropriate dosages, can greatly reduce TM mortality rates and improve satisfactory treatment outcomes.