Development and validation of a new radiographic scoring system to evaluate bone and cartilage destruction and healing of large joints with rheumatoid arthritis: ARASHI (Assessment of rheumatoid arthritis by scoring of large joint destruction and healing in radiographic imaging) study

Abstract

Sarcoidosis is a systemic granulomatous disease that can affect any organ including the nose. Nasal crusting and congestion are common nasal symptoms of sarcoidosis, whereas cases of saddle-nose deformity are rarely reported. We describe here a case of sarcoidosis that presented with saddle nose resembling relapsing polychondritis. Since sarcoidosis shares clinical features with relapsing polychondritis, the differential diagnosis of saddle nose can be challenging without a clear pathology.     

Extrapulmonary sarcoidosis of liver and pancreas： A case report and review of literature

Sarcoidosis is a chronic multisystemic granulomatous disease of unknown origin, which can involve nearly all organs. In the case of an infrequent gastrointestinal tract involvement in systemic sarcoidosis, granulomas of the liver are most commonly described while isolated pancreatic sarcoid lesions are rarely seen. We report a case of systemic sarcoidosis with exclusive extrapulmonal involvement of the liver and the pancreas in a 71-year- old white man. The diagnosis of liver involvement was confirmed by biopsy. Pancreatic surgery was needed because preoperative evaluation could not exclude pancreatic cancer and for biliary decompression. An extensive literature review of systemic sarcoidosis, focusing on reported cases with unusual presentation of sarcoidosis in the liver and the pancreas, its diagnosis, treatment, and prognosis was made.     

结节病诊治研究进展

结节病是一种病因不明的多器官受累的肉芽肿疾病,其临床表现多样,肺部和胸内淋巴结受累最为多见,容易导致误诊和漏诊.本文对结节病的诊治研究进展作一综述.     

Sarcoidosis presenting with tachy- and brady-arrhythmias.

We describe a case report of a 49-year-old man admitted to a cardiology unit with a series of arrhythmias with no initially obvious aetiology. Further assessment and the use of cardiac magnetic resonance imaging and histology allowed a diagnosis of cardiac sarcoidosis to be made. Cardiac sarcoidosis is a major cause of death in patients with systemic sarcoidosis. Cardiac magnetic resonance imaging is an additional diagnostic tool for this condition without ionizing radiation exposure.     

以肺外表现为首发症状的结节病临床分析

目的提高对首发于肺外表现的结节病的认识。方法回顾性分析了２２例肺外结节病的临床资料，所有病例均有病理检查资料。结果６例患者的首发肺外表现为周围淋巴结肿大，５例为皮肤改变，２例为眼部症状，３例为腮腺结节病，３例为骨关节炎，１例为心脏结节病，１例为肝脾肿大，１例为神经精神症状。部分肺外结节病的胸部Ｘ线检查正常，血管紧张素转化酶亦在正常范围内。结论某些以肺外表现为首发症状的结节病可能尚处于疾病的早期阶段，故早期诊断和治疗有助于缓解肺外症状，提高治愈率     

Gastric sarcoidosis: Rare revealing feature of systemic sarcoidosis

Gastric sarcoidosis is clinically manifest in less than 1% of patients with systemic disease. Stomach is the most commonly involved site. Clinical signs are non-specific.We report the case of a female patient presenting with abdominal pain with no associated symptoms. Upper gastrointestinal endoscopy showed mucosal erosions. Biopsy and histological study revealed the presence of a gastric non-caseating granuloma. No other involvement was found and infectious granulomatosis was ruled out. Patient was treated with corticosteroids with substantial improvement. One year later, the patient presented with respiratory symptoms and joint pain. Chest computed tomography showed multiple mediastinal and bilateral hilar lymphadenopathies. It also highlighted the presence of interstitial lung disease and a 1 cm pulmonary nodule. Histological examination of this nodule revealed non-caseating granuloma. Serum angiotensin converting enzyme level was elevated. Lymphocytic alveolitis was diagnosed with bronchoalveolar lavage. Systemic sarcoidosis with gastric involvement was suggested. Patient was treated with corticosteroids and developed a favourable clinical course.Isolated gastric sarcoidosis is a challenging diagnosis. Histological findings are the key to diagnosis. Other granulomatous diseases need to be excluded especially infectious diseases before establishing the diagnosis of sarcoidosis. Corticosteroids are used in treating gastric sarcoidosis, yet no guidelines exist for the treatment of this rare condition.     

Bilateral ankle edema with bilateral iritis

I report two patient presented to me with bilateral symmetrical ankle edema and bilateral acute iritis. A 42-year-old female of Indian origin and 30-year-old female from Somalia both presented with bilateral acute iritis. In the first patient, bilateral ankle edema preceded the onset of bilateral acute iritis. Bilateral ankle edema developed during the course of disease after onset of ocular symptoms in the second patient. Both patients did not suffer any significant ocular problem in the past, and on systemic examination, all clinical parameters were within normal limit. Lacrimal gland and conjunctival nodule biopsy established the final diagnosis of sarcoidosis in both cases, although the chest x-rays were normal. I do not have any financial or proprietary interest in any method or material mentioned.     

Sicca syndrome in patients with sarcoidosis

Out of 134, 12 sarcoidosis patients with symptoms of mucosal dryness as the first clinical manifestation were identified and compared with 30 consecutive unselected Sjögren's syndrome (SS) patients. Sicca manifestations were similar among the two groups, while parotid gland enlargement (PGE) was more frequently found in sarcoidosis patients (P<0.05). Patients with sarcoidosis had mainly pulmonary (P<0.001) and skin involvement (P<0.05), while SS patients presented more frequently with Raynaud's phenomenon (P<0.05). Autoantibody profile was more often found in SS patients compared to sarcoidosis (P<0.0025). The histopathological findings of minor salivary gland biopsy (MSGB) revealed noncaseating granulomas (NCG) in 58% of patients with sarcoidosis, while in SS, MSGB showed focal sialadenitis in the majority of the patients. Transbronchial lung biopsy (TBLB), which was performed in 10 sarcoidosis patients, revealed the presence of NCG in all patients. In patients with sarcoidosis and sicca symptoms as the presenting syndrome, PGE is a useful clinical finding. Searching for pulmonary involvement is a determining factor to differentiate sarcoidosis from SS. The absence of autoantibodies is another useful tool for the diagnosis of sarcoidosis. Finally, MSGB is very helpful to discriminate between sarcoidosis and SS and when MSGB is not specific, then TBLB is valuable to confirm the diagnosis.     

Delayed diagnosis of sarcoidosis is common in Brazil

OBJECTIVE:

To determine the frequency of and the factors related to delayed diagnosis of sarcoidosis in Brazil.

METHODS:

We evaluated patients with a biopsy-proven diagnosis of sarcoidosis, using a questionnaire that addressed the following: time since symptom onset and since the first medical visit; and the number and specialty of the physicians visited. We divided the patients by the timeliness of the diagnosis-timely (< 6 months) and delayed (≥ 6 months)-comparing the two groups in terms of systemic and pulmonary symptoms; extrathoracic involvement; spirometric data; radiological staging; level of education; income; and tuberculosis (diagnosis and treatment).

RESULTS:

We evaluated 100 patients. The median number of physicians consulted was 3 (range, 1-14). In 11 cases, sarcoidosis was diagnosed at the first visit. In 54, the first physician seen was a general practitioner. The diagnosis of sarcoidosis was timely in 41 patients and delayed in 59. The groups did not differ in terms of gender; race; type of health insurance; level of education; income; respiratory/systemic symptoms; extrathoracic involvement; and radiological staging. In the delayed diagnosis group, FVC was lower (80.3 ± 20.4% vs. 90.5 ± 17.1%; p = 0.010), as was FEV₁ (77.3 ± 19.9% vs. 86.4 ± 19.5%; p = 0.024), misdiagnosis with and treatment for tuberculosis (≥ 3 months) also being more common (24% vs. 7%, p = 0.032, and 20% vs. 0%; p = 0.002, respectively).

CONCLUSIONS:

The diagnosis of sarcoidosis is often delayed, even when the imaging is suggestive of sarcoidosis. Delayed diagnosis is associated with impaired lung function at the time of diagnosis. Many sarcoidosis patients are misdiagnosed with and treated for tuberculosis.     

39例结节病的诊断与治疗

报告３９例胸内结节病（Ⅰ期２２例，Ⅱ期１４例，Ⅲ期３例）的临床和病理学资料，以及治疗、随访情况。指出：①本病临床表现多种多样，应仔细分析临床和组织学资料，方能作出正确诊断。②诊断成立后，正确判断疾病活动性，是严格治疗指征的关键。③治疗过程中需密切随访，严防并发症。     

胸内结节病60例临床分析

目的　探讨结节病的临床特点及诊断方法。方法　对 1992 - 0 1～ 2 0 0 3- 0 4期间上海市胸科医院住院的经病理证实的结节病患者 6 0例作一回顾性分析 ,总结临床特点并对不同的诊断方法进行评价。结果　结节病主要累及器官以肺门及纵隔淋巴结最常见。肺功能主要为限制性通气障碍及弥散功能下降。纤支镜支气管黏膜活检和经支气管镜肺活检 (TBLB)阳性率分别为 80 %和 77 8% ,浅表淋巴结和皮肤结节活检阳性率分别是87%和 87 5 %。结论　该病临床表现非特异性。外周淋巴结活检 ,纤支镜支气管黏膜活检 ,TBLB ,简便易行 ,阳性率高 ,创伤性小 ,对结节病的诊断有较高的临床实用价值。     

结节病胸膜病变分析并文献复习

目的 分析结节病胸膜病变的临床特征，探讨鉴别诊断方法。方法回顾性分析确诊的有病理证据的结节病32例，报告以胸膜病变为主要表现的典型病例，对临床特点和鉴别诊断进行分析。检索国内文献中结节病胸膜病变的病例报告和病例总结，分析其发生率和临床特征。结果1989年至2004年共确诊胸内结节病32例，以胸腔积液为主要表现者2例（2／32，6．3％），其中1例双侧胸腔积液经内科胸腔镜检查确诊；自发性气胸1例（1／32，3．1％），经开胸肺活检确诊。胸腔积液分析为渗出液，细胞分类以淋巴细胞为主，腺苷脱氨酶（ADA）均未超过30U／L，其中1例胸腔积液血管紧张素转换酶（ACE）升高。3例在确诊前均被误诊为肺结核，接受抗结核治疗1-12个月不等。国内发表的病例总结中，结节病胸膜病变发生率为3．4％-16．7％；个案报道显示结节病胸腔积液最常被误诊为结核性胸膜炎。结论结节病胸膜病变并非罕见，临床上常被误诊。这一认识无论对于结节病的及时诊断，还是对于胸膜病变的鉴别诊断都有重要临床意义。     

Sarcoidosis pulmonar simulando una tuberculosis: importancia del signo de la galaxia en TC de tórax

Sarcoidosis and tuberculosis are two common granulomatous conditions that may share clinical and radiological presentations. The galaxy sign (sarcoid galaxy sign) is a characteristic radiological sign of pulmonary sarcoidosis on thoracic computed tomography (CT). We present the case of a patient with sarcoidosis that was initially misdiagnosed as tuberculosis, in whom the galaxy sign on CT was useful as it suggested the correct diagnosis.     

The Clinical and Radiologic Features of Nodular Pulmonary Sarcoidosis

STUDY OBJECTIVES: Nodular sarcoidosis is an uncommon presentation of sarcoidosis. Our objective was to describe the clinical characteristics of a large cohort of patients with nodular sarcoidosis. METHODS: We performed a retrospective study of patients with nodular sarcoidosis diagnosed at an urban teaching hospital over a 10-year period. RESULTS: Thirty-three patients with nodular sarcoidosis were identified. All patients were African-American. The mean age was 35 and the female-to-male ratio was 5:1. Twenty-six patients were current or former smokers. All patients had chest CT scan and/or chest radiograph evidence of pulmonary masses. Twenty-seven patients had multiple pulmonary masses/nodules and six had solitary pulmonary nodules/masses. The upper lobes were involved in 27 patients. Mediastinal lymphadenopathy and pleural-based masses were present in 30 and 20 patients, respectively. Extrapulmonary manifestations were present in 14 patients. All patients had tissue diagnosis of noncaseating granulomas with negative culture. Twenty-two patients underwent bronchoscopy with transbronchial biopsies, which were diagnostic in 19. Follow-up data were available on 27 patients: complete or nearly complete resolution of the pulmonary masses--either spontaneously or with systemic treatment--was documented for 19 patients, no change in the radiologic findings for 7 patients, and progression to pulmonary fibrosis for 1 patient. CONCLUSIONS: Nodular sarcoidosis is a rare presentation of pulmonary sarcoidosis. It usually presents with multiple pulmonary masses that tend to be peripheral and are associated with mediastinal lymphadenopathy. Bronchoscopy with transbronchial biopsies has high diagnostic yield. Despite its ominous presentation, nodular sarcoidosis has favorable prognosis.     

结节病39例误诊分析

目的探讨结节病患者误诊常见原因,以提高结节病的诊断率,减少误诊。方法回顾性分析1998年10月至2006年9月于中国医科大学附属第一医院经病理证实为结节病的98例患者中39例误诊病例的临床表现及误诊原因。结果结节病起病隐匿,临床表现缺乏特异性以及对该病的认识不足是误诊的主要原因,肺结核、肺癌、淋巴瘤是结节病主要误诊疾病。结论掌握结节病的临床特点、影像学表现,并与实验室检查结合分析是减少误诊的主要措施。     

Ductopenia related liver sarcoidosis

Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis.Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry.The occurrence of jaundice in sarcoidosis is rare;extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic.Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver-related death.We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded.The patient follow up was 8 years.Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis.A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.     

电视纵隔镜联合CD4/CD8对纵隔结核和结节病的诊断价值

目的探讨电视纵隔镜联合检测CD4／CD8在纵隔结核病和结节病诊断中的应用价值。方法回顾性分析2003年2月至2005年9月我院单纯纵隔病灶诊断不明行电视纵隔镜检查患者90例,应用免疫组化法对病理证实为结节病、结核病或不典型肉芽肿的患者57例检测CD4／CD8的表达情况。结果纵隔病灶活检确诊为结节病37例,结核病14例,淋巴瘤15例,原因不明的纵隔转移癌18例,无干酪性坏死又无典型结节细胞的肉芽肿6例,疾病确诊率为93．3％（84／90）;结节病病灶中CD4为（65±13）％,CD8为（9．4±2．6）％,CD4／CD8为7．3±1．8;结核病病灶中CD4为（41±11）％,CD8为（11．8±3．4）％,CD4／CD8为3．6±1．1,结节病病灶中的CD4／CD8明显高于结核病病灶。如果将CD4／CD8〉5作为诊断结节病的标准,则诊断结节病的准确性、特异性和敏感性分别为90．2％、85．7％和91．9％。本组病例未发生手术并发症,无死亡病例。结论电视纵隔镜检查对诊断纵隔疑难病灶的准确率高,部分病例结合CD4／CD8比值对诊断结核病和结节病有一定价值。     

La biopsie splénique échoguidée : un outil diagnostique efficient au cours de la sarcoidose ?

BackgroundHistological diagnosis of systemic granulomatosis may be difficult. The question of the best histological target remains unanswered.CaseWe report here the observation of a patient admitted in intensive care unit for severe hypercalcemia in the context of polylymphadenopathy and constitutional symptoms. Assessment of this hypercalcemia was suggestive of systemic granulomatosis. The CT (computed tomodensitometry) revealed lymphadenopathies of the mediastinum and the hepatic hilus, hepatomegaly and heterogeneous splenomegaly. At this stage, our main hypotheses were: lymphoid hematopathy, sarcoidosis, tuberculosis. An echo-guided biopsy of the spleen allowed the histological diagnosis of systemic granulomatosis suggestive of sarcoidosis, without significant complication in the course.ConclusionThis observation illustrates the efficiency and safety of spleen biopsy for the histological diagnosis of systemic granulomatosis.     

老年人结节病的临床分析

目的 提高对老年人结节病的认识.方法 回顾性分析临床确诊的13例老年结节病患者的临床资料.结果 无症状查体时发现3例,首发症状为肺内和肺外表现者各5例.胸部影像学有8例呈不典型表现.8例经纤维支气管镜检查有5例呈炎症改变.5例肺泡灌洗液中淋巴细胞比例和CD4/CD8比值增高者各3例.7例通过活检确诊.13例经治疗后均不同程度缓解病情.结论 老年人结节病临床症状和影像学表现多种多样,常不典型,明确诊断需要结合检查结果 综合分析.对有治疗指征的患者在密切监测下可给予皮质激素治疗.