结节病患者肺功能变化研究

目的：肺结节病患者肺功能测试的研究。方法：40例肺结节病患者不同时期肺功能测试结果和应用激素的回顾分析。结果：显示此类患者有限性通气功能障碍并伴有弥散功能下降和小气道功能受限,PaO2明显下降,pH升高,PaCO2也有下降的趋势。激素治疗结果显示,患者的MMF有明显的提高。其它肺功能指标如VC,RV,RV/TLC,MVV,FEV1,DLCO等都有升高的趋势。结论：VC,RV,MMF,DLCO,DLCO/VA等对诊断结节病的病情发展有直接参考价值,而MMF在糖皮质激素治疗后有显著的变化,提高MMF对结节病的治疗和预后具有动态参考价值。     

A Clinicopathologic Series of 22 Cases of Tonsillar Granulomas

Objectives Tonsils are uncommonly affected by granulomatous inflammation, often with an obscure cause. This study attempts to elucidate the nature of tonsillar granulomatous inflammation. Design Retrospective clinicopathologic review. Methods Twenty‐two cases of tonsillar granulomas diagnosed between 1940 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. H&E slides and a series of histochemical stains were reviewed, and patient follow‐up was obtained. Results There were 11 males and 11 females, aged 7 to 64 years (mean, 29.9 y). Most of the cases presented bilaterally (n = 19) with sore throat, dysphagia, and/or nasal obstruction. The clinical differential included chronic tonsillitis, tuberculosis, nonspecific infection, sarcoidosis, and a neoplasm. Histologically, the granulomas were focal and scattered, or diffuse, identified in the interfollicular zones (n = 16) and/or the germinal centers (n = 13), and occasionally associated with necrosis (n = 6). Based on histochemical and clinical follow‐up information, the etiology of the granulomas included sarcoidosis (n = 8), tuberculosis (n = 3), Hodgkin's lymphoma (n = 2), toxoplasmosis (n = 1), squamous cell carcinoma (n = 1), and no specific known cause (n = 7). Twelve patients were either alive at last follow‐up or had died with no evidence of disease (mean, 12.4 y), and 9 were either alive at last follow‐up or had died with disease (mean, 24.9 y). One patient was alive with unknown disease status (lost to follow‐up after 13.3 y). Conclusions Although a cause for tonsillar granulomas is frequently identified, a number may not develop an identifiable etiology, with the granulomas probably representing an exaggerated immune response to chronic tonsillitis. However, a careful work‐up must be conducted to exclude specific causes and avoid clinical mismanagement.     

T-cell signature cytokines distinguish pulmonary sarcoidosis from pulmonary tuberculosis

Sarcoidosis is a systemic inflammatory disorder characterized by tissue infiltration due to mononuclear phagocytes and lymphocytes and associated noncaseating granuloma formation. Pulmonary sarcoidosis (PS) shares a number of clinical, radiological, and histopathological characteristics with that of pulmonary tuberculosis (PTB). Due to this, clinicians face issues in differentiating between PS and PTB in a substantial number of cases. There is a lack of any specific biomarker that can diagnose PS distinctively from PTB. We compared T-cell-based signature cytokines in patients with PS and PTB. In this study, we proposed a serum biomarker panel consisting of cytokines from cells: T helper (Th) 1 [interferon-gamma (IFN-γ); tumor necrosis factor-alpha (TNF-α)], Th9 [interleukin (IL)-9], Th17 [IL-17], and T regulatory (Treg) [IL-10; transforming growth factor-beta (TGF-β)]. We performed the principal component analysis that demonstrated that our serum cytokine panel has a significant predictive ability to differentiate PS from PTB. Our results could aid clinicians to improve the diagnostic workflow for patients with PS in TB endemic settings where the diagnosis between PS and PTB is often ambiguous.     

良性肺结节HRCT的影像特征

目的：用HRCT评价良性肺结节。方法：35例经手术病理或临床治愈证实的良性肺结节行HRCT扫描，观察其CT表现。结果：18例结核瘤中15例呈圆形或椭圆形，有长、短毛刺及环蛋壳状钙化者分别为13和14例，10例周围有纤维条索影或卫星播散病灶，7例增强扫描4例呈无强化，3例呈典型的包膜样强化。9例炎性假瘤均发生在两肺上叶前段或下叶基底段，6例结节呈圆形或类圆形，边缘可见长毛刺和胸膜凹陷征，3例周边可见斑片影及血管集束征，7例增强扫描6例呈良性肺结节的动态强化。3例错构瘤2例检出结节边缘砂粒样钙化和中央脂肪密度。2例肺脓肿均表现为圆形或类圆形中央低密度结节，增强扫描呈典型的周边环状强化。1例硬化性血管瘤周围可见受压小血管。1例肺霉菌球呈不规则形，有长毛刺及多个小空腔，周围有血管集束征及胸膜凹陷征。1例纤维瘤呈圆形，边缘光滑，密度均匀。结论：常见及少见良性肺结节进行HRCT扫描有助其定性诊断。     

Endoscopically proven case of rapid esophagogastric variceal progression and rupture as a result of portal hypertension with liver sarcoidosis

Sarcoidosis is a multi‐systemic disease of unknown etiology that results in the development of non‐caseating epithelioid granulomas. The liver is the third most frequently involved organ after the lymph nodes and the lungs. Most cases of liver sarcoidosis do not present with symptoms and involve minimal liver dysfunction, but some cases display progression to portal hypertension and liver cirrhosis, and finally to liver failure. The mechanism and the risk of progression in liver sarcoidosis are still unknown because of the diagnostic difficulty associated with this condition, and because follow‐up examinations can only be done in an invasive manner. Here, we present an informative case of liver sarcoidosis with rapid progression of esophagogastric varices. Four months prior to the definitive diagnosis, no signs of varices were observed on endoscopy, and developmentof esophagogastric varices, rapid progression, and eventual rupture occurred in a short period of time. A liver biopsy, carried out after endoscopic sclerotherapy, revealed that granulomas primarily affected the portal area without fibrotic and cirrhotic changes, which is considered a primary cause of portal hypertension and esophagogastric varices. Following the liver biopsy, the patient was given systemic steroids and is currently receiving outpatient care. Thus, we should consider the possibility that liver sarcoidosis, even in the absence of cirrhotic changes, can cause serious events such as esophagogastric variceal rupture following rapid progression as a result of portal hypertension.     

A case of nodular-type muscular sarcoidosis: findings of imaging,histopathology, and polymerase chain reaction

Abstract

We report a case of nodular-type muscular sarcoidosis with no systemic symptoms. Thallium-201 scintigraphy showed intense uptake in the muscular lesion mimicking malignant soft tissue tumor. Magnetic resonance imaging (MRI) demonstrated characteristic signal patterns of peripheral high intensity with central low intensity (“three stripes” pattern). Microscopy revealed sarcoid granuloma with typical histopathological characteristics. Propionibacterium acnes was detected on polymerase chain reaction analysis of the excised tissue.     

Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 2 of a 2-Part Series

Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies. The detection of infiltrative cardiomyopathies, particularly primary and secondary forms of iron overload, as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via disease-specific therapies.     

More Than a Rash

Skin disorders are one of the most common complaints seen in primary care offices. This can be intimidating and frustrating for providers due to the wide variety of possibilities. Skin lesions that are difficult to manage or concerning are often referred to dermatology to determine the potential etiology. The following is a case study of an elderly woman who presented with a persistent rash for the past 6 months despite clinically appropriate treatment. This case study describes the interdisciplinary care implemented for diagnosis, treatment, and follow up-care.