THE SPECTRUM OF NEUROLOGICAL INVOLVEMENT IN SJOGREN'S SYNDROME

Sixty-three unselected consecutive patients with primary Sjögren'ssyndrome (pSs) were prospectivelyevaluated for evidence of neurologicalmanifestations. Seventeen had a mild sensory or mixed neuropathy.Two of these plus one more patient had trigeminal neuropathy.One had pure motor neuropathy. whereas another eight had latentmotor neuropathy. None volunteered neurological complaints.Two more patients had symptomatic unilateral carpal tunnel syndrome.Severe mononeuritis multiplex and symptomatic symmetrical distalneuropathywere seen in two patients with vasculitis. One patientwith a history of hypertension and on subjective sicea complaints,had a mild cerebrovascular accident and objective evidence ofchanges compatible with pSs. The study suggests that peripheralneurological involvement is relatively common and benign inthe majority of pSs individuals, whereas central nervous system(CNS) disease must be rare. KEY WORDS: peripheral neuropathy, Mononeuritis multiplex, Carpal tunnel syndrome, Central nervous system, Nerve conduction studiesSicca syndrome     

THE ARTHROPATHY OF SJOGREN'S SYNDROME

The clinical course of 48 patients with primary SS has beenreviewed with particular reference to the articular manifestations.The incidence of arthritis and/or arthralgia was 54%. In a thirdof these patients it was a presenting feature and preceded siccasymptoms. The arthropathy tended to be polyarticular, the mostfrequent joint involved being the knee. It was symmetrical in55 % of cases. Joint symptoms or signs were intermittent, lastingless than a month in 55 % of cases. The acute onset of purpuricvasculitis was associated with an acute arthritis in four outof the nine patients with such a vasculitis. Joint deformitywas unusual, ulnar deviation occurring in only six patients.Hand X-rays obtained from primary SS patients revealed evidenceof joint erosions in 33% of PIP joints, 27% of MCP joints and12% of wrist joints. KEY WORDS: Sjögren's syndrome, Arthritis     

PROGRESSION OF SIALADENITIS IN SJOGREN'S SYNDROME

An analysis of progression of sialadenitis in patients withprimary and secondary SS has been performed. For this purposepatients were prospectively followed and evaluated with respectto stimulated whole salivary secretion and morphology of labialsalivary gland biopsies. Twenty-one patients with primary SSand 18 with secondary SS were followed for a mean of 39 ±20 months (range 11–112 months). During this observationperiod the lymphocytic infiltration in minor salivary glands,measured as focus score, increased in 14/21 (67%) patients withprimary SS and in 14/18 (78%) patients with secondary SS. Altogetherthere was a statistically significant increase in focus scorein both primary and secondary SS, but no reduction in salivaryproduction. Consequently, no correlation between changes infocus score and stimulated salivary secretion was found in eitherprimary or secondary SS. KEY WORDS: Salivary glands, Salivary flow, Histopathology     

DEVELOPMENT OF HODGKIN'S DISEASE IN THE COURSE OF PRIMARY SJOGREN'S SYNDROME

We report two patients with a definite diagnosis of primarySjögren's syndrome who developed Hodgkin's disease. Clinicaland laboratory features of this transformation comprised prolongedfever, the appearance of lymphadenopathy together with lossof serum autoantibodies and a reduction in serum gammaglobulinlevels. We know of only one well documented case of such anassociation. From these observations, it seems reasonable toinclude Hodgkin's disease in the clinical spectrum of the lymphoproliferativedisorders that may occur in the course of primary Sjögren'ssyndrome. KEY WORDS: Sjögren's syndrome, Hodgkin's disease, Lymphoma, B-cell     

A PRELIMINARY STUDY ON THE INTERFERON-{alpha} TREATMENT FOR XEROSTOMIA OF SJOGREN'S SYNDROME

We studied the therapeutic effect of interferon-     

HETEROGENEITY OF THE POLYCYSTIC OVARY SYNDROME: CLINICAL, ENDOCRINE AND ULTRASOUND FEATURES IN 556 PATIENTS

This paper reports an analysis of the clinical, endocrine and ultrasound data within a population of 556 patients with ultrasound-diagnosed polycystic ovaries. Compared with those not so affected, hirsutism was associated with a higher mean serum testosterone concentration, infertility was associated with higher mean gonadotrophin concentrations, obesity was associated with a higher mean serum testosterone concentration, hyperprolactinaemia was associated with a lower mean serum testosterone concentration and smaller ovaries, alopecia was associated with lower mean serum LH and testosterone concentrations, and acanthosis nigricans was associated with obesity and a raised mean serum testosterone concentration. The heterogeneity illustrates the limitations in the use of specific clinical or endocrine criteria as requirements for the diagnosis of the polycystic ovary syndrome.     

SJOGREN'S SYNDROME: A STUDY OF ITS NEUROLOGICAL COMPLICATIONS

A detailed retrospective study of 105 patients with Sjoögren'ssyndrome (50 primary and 55 secondary cases), showed that 31had vasculitis and 18 had neurological abnormalities which afterfull investigation were not attributable to other causes. Mostof the neurological symptoms were mild and when found in patientswith secondary Sjoögren's syndrome were more characteristicof the underlying autoimmune rheumatic disease. We found nosignificant association between the frequency of either vasculitisor any autoantibodies and the presence of neurological disease,but did confirm a significant association between vasculitisand the presence of antibodies to extractable nuclear antigens.We therefore question whether severe and relapsing neurologicaldisease is common in patients with Sjoögren's syndrome. KEY WORDS: Sjoögren's syndrome, CNS disease     

原发性干燥综合征眼科诊断试验的评价

目的:在我国患者中重新评价原发性干燥综合征(PSS)眼科诊断试验的临床价值。方法:用Schirmer试验、BUT试验和角膜荧光素活体染色试验。在112例PSS患者和185例对照者中,评估上述试验的敏感度和特异度及其主要影响因素。结果:Schirmer、角膜荧光素活体染色和BUT试验的特异度分别为43.8％、93.5％和31.4％,敏感度分别为91.7％、50.0％和87.5％。统计分析显示Schirmer试验和BUT试验的特异度与年龄呈负相关(P<0.001),而敏感度与年龄呈正相关(P<0.05)。在55岁以上的患者,除角膜荧光素活体染色试验外各项试验的诊断价值有限。按目前我国诊断KCS的标准,上述三项试验中两项或两项以上异常,诊断的敏感度和特异度分别为84.8％和49.7％。结论:有持续性眼干患者并Schirmer试验(<5mm/5min)和(或)角膜荧光素活体染色试验阳性可明显提高临床诊断的敏感度(79.5％)和特异度(97.8％)(P<0.001)。     

LIVER INVOLVEMENT IN PRIMARY SJOGREN'S SYNDROME

Three hundred patients with primary Sjögren's syndrome(pSS) were investigated for liver involvement using clinical,biochemical, immunological and histological data. Seven percent of patients showed evidence of liver disease eithersubclinical (2%) or asymptomatic (5%) with elevated liver enzymesIn 6.6 % of patients antimitochondrial antibodies (AMA) weredetected by immunofluorescence and 27% of pSS patients showedantibodies to pyruvate dehydrogenase (a-PDH) using ELISA. AMA-positivepatients were further investigated with transcutaneous liverbiopsy. Ninety-two per cent of patients with AMA showed liverinvolvement with features of chronic cholangitis similar tostage I primary biliary cirrhosis It is concluded that liverinvolvement in pSS patients is rare and subclinical with histologicalfeatures predominantly of stage I primary biliary cirrhosisAMA is the most sensitive indicator of underlying liver pathologyin pSS patients KEY WORDS: Sjögren's syndrome, Antimitochondrial antibodies, Primary biliary cirrhosis     

老年人甲状腺功能亢进的临床特点

目的探讨老年人甲状腺功能亢进(甲亢)的临床特点。方法对1994年6月～2001年12月期间住我院的44例≥60岁老年甲亢患者的临床资料进行回顾性分析,并与随机抽取的同期住院50例中青年甲亢患者进行对比研究。结果老年甲亢患者临床症状不典型者较中青年组多;淡漠、嗜睡、精神症状、呕吐和粘液性水肿仅见于老年组;老年组中有消化系统症状者较中青年组多;心血管系统症状老年组以房颤等心律失常、心衰、心脏增大为主,心率>90次/分者较中青年组少;老年组中突眼者较中青年组少,而贫血、糖代谢异常者较之多见。以上结果,两组比较均有显著性差异(P<0.05)。老年组的血清FT3值明显低于中青年组(P<0.01);而两组血清FT4值、TSH值比较无显著性差异(P>0.05)。结论老年人甲亢的临床特点:(1)临床症状不典型者多,尤以淡漠、精神症状、嗜睡、呕吐、粘液性水肿为特殊;(2)消化系统症状以食欲减退等为多;(3)房颤等心律失常、心衰、心脏增大者多,而心率>90次/分者较中青年少;(4)突眼者少;(5)贫血者多;(6)糖代谢异常者多;(7)血清FT4值、TSH值变化近似中青年组,而FT3值虽升高,但幅度较中青年组明显低。     

THE DIAGNOSTIC VALUE OF SALIVARY GLAND SCINTIGRAPHY IN PATIENTS SUSPECTED OF PRIMARY SJOGREN'S SYNDROME

The diagnostic value of salivary gland scintigraphy in patientssuspected of having primary Sjögren's syndrome (SS) wasstudied in 149 consecutive patients who presented with jointand/or muscle complaints in combination with dryness of themouth and/or eyes and in 20 control subjects. The diagnosisprimary SS could be established in 26 of these patients. Salivarygland scintigraphy scored by means of analogue pictures wasabnormal in 19 out of 26 patients with primary SS but abnormalscintigrams were also found in 57 of the 123 patients withoutprimary SS and in five of the controls. This resulted in a positivepredictive value of an abnormal salivary gland scintigram of25% and a negative predictive value of a normal investigationof 90%. From the high number of false positive test resultsin the patient population studied it is concluded that salivarygland scintigraphy has only a limited discriminatory value forthe diagnosis of primary SS. KEY WORDS: Connective tissue disease, Sicca complaints, Diagnosis     

Cushing综合征的酮康唑治疗

应用酮康唑(ketoconazole)治疗8例Cushing综合征,剂量0.4～0.8g/d,平均疗程45天。观察治疗前后患者的体重、血压、血糖、血电解质、血浆皮质醇及24小时尿17羟、17酮类固醇等项指标的变化和药物的不良反应。结果表明:治疗后患者的体重、收缩压、舒张压、血糖、血浆皮质醇和尿17羟、17酮类固醇水平均较治疗前显著下降(P<0.05～0.001);血钾虽有所上升,但无统计学意义。治疗期间未见有严重副作用,提示此药对各型Cushing综合征具有较肯定的疗效。     

RAYNAUD'S PHENOMENON IN PRIMARY SJOGREN'S SYNDROME

Forty-five consecutive primary Sjogren's syndrome (SS) patientswere divided into two subgroups according to the presence (n= 30) of Raynaud's phenomenon (RP). Non-erosive arthritis wassignificantly more frequent in the patients with RP than inthose without. RP was not associated with HLA-DR4 in primarySS patients KEY WORDS: Joint erosions, Sicca syndrome, Rheumatoid arthritis, Tissue type     

'SERONEGATTVE' SJOGREN'S SYNDROME MANIFESTED AS A SUBSET OF CHRONIC FATIGUE SYNDROME

We determined the extent to which chronic fatigue syndrome (CFS)patients with sicca symptoms fulfil the diagnostic criteriafor Sjögren’s syndrome (SS). Three sets of diagnosticcriteria for SS, formulated by the Japanese, Europeans and Fox,were used. One-third of the CFS patients with sicca symptomsfulfilled the diagnostic criteria for SS. However, they were‘seronegative’, differing from the ordinary primarySS. KEY WORDS: Chronic fatigue syndrome, Sj     

CUSHING''S SYNDROME IN PREGNANCY: THE TIMING OF DEFINITIVE TREATMENT

Cushing's syndrome in pregnancy is rare but associated with a high fetal loss rate, premature labour and excessive maternal morbidity. There has been controversy regarding the safety and efficacy of surgical treatment during pregnancy. We describe two further cases, both due to adrenal adenomas, in whom the diagnosis was made at 28 and 31 weeks gestation. Both cases suffered from severe myopathy. The first case was not treated during pregnancy and developed wound and urinary infections after caesarean section and subsequent adrenalectomy. An incisional hernia in the caesarean section scar has been repaired twice. The second had an adrenalectomy when 29 weeks pregnant with rapid resolution of the features of Cushing's syndrome, particularly the myopathy, and had an uneventful vaginal delivery. The second case, and a review of those previously described, indicates that surgical treatment during pregnancy is safe and significantly reduces fetal losses, premature labour and maternal morbidity.     

LEPROSY NEPHROPATHY: A REVIEW OF CLINICAL AND HISTOPATHOLOGICAL FEATURES

Leprosy is a chronic disease caused by Mycobacterium leprae, highly incapacitating, and with systemic involvement in some cases. Renal involvement has been reported in all forms of the disease, and it is more frequent in multibacillary forms. The clinical presentation is variable and is determined by the host immunologic system reaction to the bacilli. During the course of the disease there are the so called reactional states, in which the immune system reacts against the bacilli, exacerbating the clinical manifestations. Different renal lesions have been described in leprosy, including acute and chronic glomerulonephritis, interstitial nephritis, secondary amyloidosis and pyelonephritis. The exact mechanism that leads to glomerulonephritis in leprosy is not completely understood. Leprosy treatment includes rifampicin, dapsone and clofazimine. Prednisone and non-steroidal anti-inflammatory drugs may be used to control acute immunological episodes.     

MAJOR UPPER AIRWAYS OBSTRUCTION ASSOCIATED WITH SJOGREN'S SYNDROME: A CASE REPORT AND LITERATURE REVIEW

This short report describes the development of life-threateningupper airways obstruction in a patient with primary Sjögren'ssyndrome (PSS), and reviews the published literature on thisunusual development. KEY WORDS: Sicca syndrome, Parotid glands, Proptosis, Tracheobronchial obstruction     

A CASE OF PITUITARY DEPENDENT CUSHING''S DISEASE WITH CLINICAL AND BIOCHEMICAL FEATURES OF THE ECTOPIC ACTH SYNDROME

A case of atypical pituitary dependent Cushing's disease is reported. The patient presented with clinical symptoms similar to those of the ectopic ACTH syndrome; notably a marked hypokalaemic alkalosis, widely fluctuating plasma cortisol levels, greatly elevated plasma ACTH levels, and failure to suppress both plasma cortisol and ACTH levels following high dose oral dexamethasone. However, a large aggressive pituitary tumour was detected by skull X-ray and computed tomography. Removal of the pituitary tumour led to full remission of the patient's Cushing's syndrome. Pro-opiomelanocortin (POMC) related peptides in the plasma and tumour tissue extract of this patient have been characterized by gel-filtration and Concanavalin-A Sepharose affinity chromatography, indicating processing of POMC in a manner more usually associated with ectopic tumours.     

兰索拉唑治疗十二指肠溃疡的临床观察

为验证第二代质子泵抑制剂兰索拉吐治疗十二指肠溃疡的疗效，对经胃镜检查诊断的治疗活动期十二指肠溃疡患者３８例作了观察，并与奥美拉唑组３４例进行了对照。结果两组十二指肠溃疡４周的愈合率分别为９７．４％和９１．２％，有效率分别为１００％与９７．１％（Ｐ＞０．０５）；疼痛消失率两组分别为１００％和９３．５％（Ｐ＞０．０５），而３天内疼痛消失率分别为７４．３％和５１．６％（Ｐ＜０．０５）。兰索拉唑治疗过程中未发现有副作用。提示兰索拉唑是治疗十二指肠溃疡有效的和安全的药物。