Rheumatic fever--a vignette

SIR, A 12-yr-old boy presented with 7 weeks of arthralgia, affectingthe knees, cervical spine and small joints of both hands, andpain in the palms of     

Rheumatic diseases and chronic myelogenous leukemia, presentation of four cases and review of the literature

We report four patients with rheumatic disease (RD) and chronic myelogenous leukemia (CML). In two patients with Behcet’s disease (BD) and rheumatoid arthritis (RA), CML developed after RD, in two patients with diffuse cutaneous systemic sclerosis and spondyloarthropathy, RD was diagnosed after CML. A variety of interactions have been described between hematological malignancies and RD. Nevertheless, few cases of RD have been documented associated with CML. It is unclear whether the development of CML in patients with RD and RD development after CML occurs by chance alone, is due to the underlying disease, or is facilitated by drugs. Whatever the cause is, it should be kept in mind that CML may develop in the course of RD and RD may be seen in CML patients.     

Clinicopathological review of Japanese cases with neoplastic angioendotheliosis]

The patient was a 76-year-old female who had been referred to our hospital because of fever of unknown origin on October 15, 1987. On admission, the body temperature was 38.6 degrees C and atonic palsy of the left upper limb was noted. Abnormal laboratory findings included CRP5+, an increase in LDH, Hb 7.9 g/dl. The cause of the fever could not be identified. The fever did not respond to various treatment. The patient developed DIC in late October and died on November 5. In autopsy histological examination revealed tumor cells in the vessels of the generalized organs. A diagnosis of neoplastic angioendotheliosis (NAE) and immunohistologically B lymphoma was made. We reviewed the literature on 37 Japanese cases of NAE. The cases, consisting of 19 males and 18 females, were aged 37-87 years with a median value of 60 years. The symptoms observed during the course were most frequently mental or neurological symptoms and fever, and rash was uncommon. Laboratory findings were non-specific and biopsy was needed for definitive diagnosis. By autopsy, lesions were noted more frequently in the brain, kidneys, and lungs, and the findings in the skin were indeterminate. These observations suggest that when NAE should be considered, kidney, lung or skin biopsy should be performed for definitive diagnosis.     

Rheumatic diseases in advanced age--psychological aspects]

This paper discusses the importance of cognitive representation and individual patterns of behavior for coping with chronic disease. Chronically ill patients differ very much in their psychic situation. Biographical and situational factors and the future perspective contribute to these interindividual differences. Diagnostic and therapeutical tools are discussed from a psychological perspective.     

Beaver fever--a rare cause of reactive arthritis

Giardia lamblia infection is rarely associated with adult reactive arthritis. We report the first North American case and review the pediatric and adult literature to date. Antimicrobial treatment is essential to eradicate the parasite and control the arthritis.     

Six cases of thymic carcinoma: a clinical review]

Thymic carcinoma is rarer than thymoma and carries a very poor prognosis. No standard treatment has yet been established. Chemotherapy and radiation therapy are usually given to patients in whom surgery is not indicated. We clinically review six patients with thymic carcinoma (three men and three women) who were admitted to our hospital from 1992 through 2002. Their ages ranged from 56 to 81 years (median 70.5). Four patients had chest pain, two were unable to swallow, and in one, who was asymptomatic, the tumor was detected by chance. Histologically, five cases were squamous cell carcinomas, and one was a lymphoepithelioma-like carcinoma. Four of the six patients had distant metastasis at diagnosis. Only one patient was able to undergo surgery, two received radiation therapy alone, and three were administered chemoradiation. The response to the chemoradiation was progression of the disease in two patients and no change in one. The response to radiation therapy was partial response in one patient and no change in one. In summary, chest pain is the most common symptom of thymic carcinoma. A symptomatic case suggests a poor prognosis resulting from the clinical behavior of thymic carcinoma, such as early distant metastasis or direct invasion of surrounding organs. Histologically, squamous cell carcinoma is the most common, and thymic carcinoma appears to be generally resistant to chemotherapy and radiotherapy.     

Primary gallbladder carcinoma. A review of 41 cases]

We present a retrospective study of primary carcinoma of the gallbladder with a total of 41 cases over a period of 11 years. In the majority of cases the clinical presentation (cholelithiasis, choledocholithiasis); there were only 7 cases of suspected diagnosis, mainly through the use of new diagnostic techniques (echography, computerized tomography). The classification used is that described by Nevin (1976) of affection of gallbladder wall and adjacent tissues. The conclusions of our study are as follows: a) primary neoplasias of the gallbladder are aggressive tumours carrying a poor prognosis aggravated usually late diagnosis due to the scarce symptomatology, b) given the high incidence of cholelithiasis, it is advisable to perform routine cholecystectomy, c) survival is related to the histological grading of the tissues.     

Clinicopathologic review of 41 cases of pancreatic mucinous cystic neoplasms]

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms are included in mucin-producing pancreatic tumors. The reports about IPMN are not uncommon but those about the mucinous cystic neoplasms are relatively few. The aims of this study were to define the natural history of resected mucinous cystic neoplasms of the pancreas and to identify the findings which suggest malignancy. METHODS: The authors retrospectively evaluated the clinical outcomes of 41 patients with mucinous cystic neoplasms who were surgically resected at Asan Medical Center between 1995 and 2004. RESULTS: Women (n=33) were more frequently affected than men (n=8). Thirty three patients (80.6%) had adenoma, 1 (2.4%) borderline malignancy, 1 (2.4%) carcinoma in situ, and 6 (14.6%) invasive mucinous cystadenocarcinoma. The most frequent symptom was abdominal pain (39%). About half of the enrolled patients were asymptomatic. Unilocular type (79%) was more frequent than the multilocular type (21%) on gross morphology. The tumor size of invasive mucinous cystic neopolasms was larger than that of non-invasive mucinous cystic neoplalsms (p=0.01). Abdominal pain was more frequent in invasive mucinous cystic neoplasms (p=0.026). On gross morphology, mural nodules were detected in 4 of 6 patients with invasive mucinous cystic neoplasms. However, they were not detected in any patients with non-invasive mucinous cystic neoplasms. Recurrence developed in none of the 35 patients with non-invasive mucinous cystic neoplasms, however 2 of the 6 patients with invasive mucinous cystic neoplasms died within 5 years. CONCLUSIONS: Clinical predictors of invasive mucinous cystic neoplasms are suggested to be tumor size and abdominal pain. The prognosis of the non-invasive mucinous cystic neoplasms is excellent when curative resection is performed.     

Rheumatic associations of autoimmune thyroid disease: a systematic review

Clinical Rheumatology - To investigate specific disease patterns in the rheumatic manifestations associated with autoimmune thyroid disease (AITD) through a systematic literature review. We...     

风湿性疾病合并骨髓增生异常综合征九例临床分析

目的 了解风湿性疾病合并骨髓增生异常综合征（MDS）的临床特点及可能的发病机制。方法 对住院确诊的9例风湿性疾病合并MDS的临床特征进行统计分析。结果 9例MDS患者中有4例诊断MDS前已有风湿病史，9例确诊MDS时均有活动期风湿病表现，伴有红细胞沉降率增快、不同程度血清免疫球蛋白升高（主要为IgG和IgM）及多种不同的自身抗体阳性，其中2例合并类风湿关节炎（RA）和RA＋抗磷脂综合征（APS），4例系统性红斑狼疮（SLE）和SLE＋多发性肌炎（PM），1例成人斯蒂尔病，1例伴有急性关节炎、皮肤血管炎等风湿病表现，1例伴有多浆膜腔积液、蛋白尿、血清补体下降、Commb’s试验阳性等狼疮样表现。结论 风湿性疾病合并MDS时多有不同的活动期风湿病表现．患者同时存在免疫功能紊乱和造血功能异常，可能由于免疫功能失调，如T、B细胞功能异常、高丙种球蛋白血症、出现自身抗体等引起不同的风湿性疾病表现，说明MDS与风湿性疾病之间存在内在的关联。     

Hepatic brucelloma: 2 cases and a review of the literature]

We report two new cases of hepatic brucelloma in addition to the 22 previously reported cases in the literature. Our analysis of these cases reveals certain characteristics. Hepatic brucelloma is a rare localization that follows previously undetected acute brucellosis. Brucelloma is a result of caseification of a granulomatous reaction induced by persistent Brucella in macrophages. Clinical manifestations can mimic malignant liver tumors or pyogenic, amebic liver abscess. Diagnosis is based on the association of characteristic imaging features (central calcification and peripheral necrotic areas), positive serology and hepatic granulomas. Brucella is rarely isolated in the blood or liver. Awareness of this clinical variant can prevent unnecessary laparotomy. Treatment should begin with rifampicine (900 mg per day) and doxycyclin (200 mg per day) for 3 months. If medical treatment is unsuccessful, percutaneous or surgical drainage should be performed. A cure should be achieved in all cases.