Complete heart block in lupus.

Conduction abnormalities are uncommon in adult patients with lupus. We present a young woman with recurrent syncope caused by third-degree atrio-ventricular block as the initial manifestation of lupus and review 31 additional cases of systemic lupus erythematosus patients that have been described previously with complete heart block. Heart blocks occurred almost exclusively in females. The median age was 37 years. In 24 cases heart blocks were diagnosed in patients with established lupus. In only five patients, including the patient presented here, heart blocks were diagnosed before the lupus diagnosis. Syncope was the most common presenting symptom of heart block. Electrocardiographic findings prior to heart block episodes were reported in 17 cases: eight had normal findings, but nine had already variant forms of atrioventricular or intraventricular conduction defects. Anti-nuclear antibody tests were reported in 25 cases and were all positive. Anti-DNA antibodies were also common and were positive in 16 of 19 cases (84%). Anti-La and anti-Ro antibodies were less common (13% and 35%, respectively). Three patients died, all prior to 1975. Heart block resolved in 10 cases. Follow-up was reported in four of these cases and heart block recurred in three of them. A permanent pacemaker was the eventual treatment in 22 cases. The etiology of lupus-associated complete heart block is not clear. It is probably variable, possibly related to effects of autoantibodies reacting with the conduction system, myocardial disease and adverse effects of antimalarials. Insertion of a permanent pacemaker seems to be the preferable method of treatment.

Effect of biopsy length on the rate of positive temporal artery biopsies.

OBJECTIVE: To investigate the relationship between temporal artery biopsy (TAB) length and the diagnostic sensitivity for giant cell arteritis (GCA). METHODS: TAB pathology reports were reviewed for histological findings and formalin-fixed TAB lengths. The patient's charts were reviewed for clinical data. TAB was considered positive if there was a mononuclear cell infiltrate in the vessel wall. Biopsy-negative GCA was diagnosed when patients fulfilled the American College of Rheumatology classification criteria, in addition to favorable rapid response to steroid therapy. Patients were divided into 3 groups according to the clinical and histological features: Biopsy-positive GCA, biopsy-negative GCA, and no GCA. RESULTS: 305 TAB reports of 173 individuals were reviewed. When only GCA patients TAB-positive and TAB-negative were considered, TAB in the biopsy-positive patients was significantly longer than in biopsy-negative cases (p=0.008). The rate of positive biopsies was only 19% with TAB length of 5 mm or less, but increased to 71-79% with TAB lengths of 6-20 mm, and to 89% when TAB length was longer than 20 mm. Only 3% of positive biopsies were 5 mm or shorter, compared to 27% of TAB in biopsy-negative GCA cases (p<0.001). CONCLUSION: TAB with post-fixation length shorter than 5 mm carries an increased biopsy-negative rate therefore longer TAB length is required for accurate diagnosis. Increasing post-fixation TAB length beyond 20 mm may further increase the rate of positive biopsies, although data were insufficient in that regard.

Giant cell arteritis: intensity of the initial systemic inflammatory response and the course of the disease.

OBJECTIVE: Giant cell arteritis (GCA) has a variable course. We assessed whether intensity of initial systemic inflammatory response (ISIR) can predict the course of GCA. METHODS: Charts of 130 GCA patients were reviewed. ISIR intensity at presentation was determined by 5 parameters of inflammation: sedimentation rate >100 mm/h, thrombocytosis >400,000/microl, hemoglobin <11 g/dl, leukocytosis >11000/microl, and fever >37.5 degrees C. Patients were divided into 3 groups according to ISIR intensity: strong (4-5 parameters present, n=24), moderate (2-3 parameters, n=55) and weak ISIR (0-1 parameter, n=51). RESULTS: There were no significant differences between these groups regarding mean age, female:male ratio and the initial prednisone dose. At 1 year, 75% of patients in the strong ISIR group required >5 mg/d of prednisone, compared to 54% and 37% of patients with moderate or weak ISIR, respectively (p=0.015). Disease flares were more common in patients with strong ISIR during a 3-year period, compared to patients with moderate or weak ISIR (77%, 67% and 43%, respectively, p=0.013). Only 33% of patients with strong ISIR were able to discontinue steroids after 3 years, compared to 49% and 77% of patients with moderate and weak ISIR, respectively (p=0.003). CONCLUSION: GCA Patients with strong ISIR have prolonged disease course with more flares, requiring higher steroid doses. ISIR intensity should be taken into consideration when planning studies evaluating potential steroid-sparing agents, as response to treatment may vary in patients with different ISIR intensities.

Negative temporal artery biopsies: eventual diagnoses and features of patients with biopsy-negative giant cell arteritis compared to patients without arteritis.

OBJECTIVE: Characterize patients with negative temporal artery biopsies in regard to their eventual diagnoses, and to find features that would differentiate biopsy-negative GCA from non-GCA patients. METHODS: 58 patients with negative biopsies were included. Patients' data and final diagnoses were obtained from medical records. Biopsy-negative GCA was diagnosed when the American College of Rheumatology classification criteria were met, symptoms improved within 3 days of corticosteroid therapy, and no other condition relevant to the patient's symptoms diagnosed during a follow up of 6 months. RESULTS: Biopsy negative GCA was diagnosed in 11 cases (19%). "Isolated" polymyalgia rheumatica was eventually diagnosed in 5 patients (9%). Altogether, rheumatologic conditions were diagnosed in 23 cases (40%). Other patients (60%) had various hematologic, neurologic-ophthalmic, infectious and malignant disorders. Patients with biopsy-negative GCA were older than non-GCA cases, 81.7+/-6.2 and 74.8+/-8 years, respectively (p=0.05). Headaches were more common in biopsy-negative GCA patients: 91% of them presented with headaches, compared to only 40% of non-GCA patients (p=0.005). Thrombo-cytosis was more common in patients with biopsy-negative GCA compared to non-GCA patients (73% and 19%, respectively, p=0.001). Other clinical and laboratory parameters did not differ significantly between the two groups. CONCLUSIONS: 19% of patients with negative temporal artery biopsies were eventually diagnosed as GCA. Older age, headache and thrombocytosis were more common in that group. These features may help in the diagnostic approach in cases with negative biopsies.

The incidence of giant cell arteritis in Jerusalem over a 25-year period: annual and seasonal fluctuations.

OBJECTIVE: Giant-cell arteritis (GCA) incidence is reported to be rising. A cyclic pattern of annual incidence rates and seasonal variations were reported by several groups. However, such fluctuations were not observed by others. We examined both annual and seasonal rates of GCA over a period of 25 years in Jerusalem. METHODS: Charts of all patients diagnosed as GCA between 1980-2004 were reviewed. In 170 cases GCA was biopsy-proven. Thirty-six additional cases were included as they met the American College of Rheumatology GCA classification criteria. Data on the Jerusalem population throughout the study period was collected from the annual publications of the Israel Bureau of Statistics. Age- and sex-specific incidence rates per 100000 population aged>or=50 were calculated. RESULTS: For the whole period, the average age-adjusted incidence rate was 11.3 per 100000, and 9.5 for the biopsy-positive cases. The female: male ratio was 1.4:1. Cyclic fluctuations of GCA incidence with 3 distinctive peaks, 8-10 years apart, were observed. Altogether, there was no apparent increase in GCA incidence during this period. Seasonal variations were observed: in 192 patients we were able to estimate the time of onset of GCA symptoms. It showed a peak in the months of May and June, with the number of patients being twice as expected for this period (p<0.001). CONCLUSION: GCA onset was more common in late spring and early summer, and fluctuations in GCA annual incidence with 3 distinctive peaks were observed during a 25-year period. These suggest infectious or other environmental etiology, however thus far no such agents were proven.

Perceived efficacy among patients of various methods of complementary alternative medicine for rheumatologic diseases.

OBJECTIVE: The purpose of this cross-sectional survey was to obtain and analyze data on self-perceived efficacy of different types of complementary alternative medicine (CAM) by patients with various rheumatologic conditions. METHODS: Patients followed in rheumatology outpatient clinics were screened for the use of CAM. Patients reporting the use of CAM were asked to participate in face-to-face structured interviews, specifying the various CAM types they used, and grading their subjective impression of efficacy of each CAM type on a scale of 1-10. RESULTS: 350 consecutive patients were screened and 148 reported using CAM. In general, homeopathy and acupuncture were the most commonly used CAM types (44% and 41% of the CAM users, respectively). The mean number of different CAM methods used by a CAM user was 1.9 +/- 1.1. Patients with fibromyalgia used significantly more CAM methods (2.7 +/- 1.4, p = 0.005). On patients' self-perceived efficacy scale of 1-10, the mean score of the whole group was 5.3 +/- 3.2. Acupuncture and homeopathy achieved significantly higher self-perceived efficacy scores in CAM users with spondylo-arthropathies and osteoarthritis, respectively, when compared to some of the other disease groups. Satisfaction was lowest among CAM users with rheumatoid arthritis, vasculitis and connective tissue diseases. CONCLUSION: In general, CAM users were less than moderately satisfied with self-perceived-efficacy of CAM therapies. However efficacy of specific CAM methods differed significantly among patients in different disease groups.

A pregnant woman with hepatitis A and Guillain-Barré.

Guillain-Barré syndrome (GBS) is often preceded by an infectious disease. A case of GBS after hepatitis A in a pregnant woman is described. The patient was treated with intravenous immunoglobulin and had full recovery with no neurologic sequelae. She gave birth in term to a healthy baby. This is the first reported case in the English literature of a triple condition of hepatitis A, GBS, and pregnancy.

Infective endocarditis associated with upper endoscopy: case report and review.

We describe a 73-year-old male who developed native valve infective endocarditis due to Staphylococcus capitis as a consequence of repeated oesophageal dilatation. The patient was treated with appropriate antibiotic therapy but his condition deteriorated and he died. We review 10 previously reported cases of infective endocarditis associated with upper endoscopy and discuss the issue of prophylaxis of high risk patients prior to upper endoscopy involving various invasive procedures, e.g. biopsy, dilatation and sclerotherapy.

Circumcision complications and indications for ritual recircumcision--clinical experience and review of the literature.

The aim of this work is to review the complications of circumcision in the literature of medicine and Jewish law. One of the problems discussed in Jewish law concerns a remnant of the prepuce covering the corona, thus causing the circumcision to be incomplete. There is a controversy in Jewish law sources regarding the definition of the corona--whether it is all the glans penis, or only the cord surrounding the base of the glans. During the last 8 years, our center has been consulted about recircumcision for 60 children and about treatment for another group who had developed complications due to circumcision, often as a result of "nonprofessional" circumcision.