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.

Severe ASIA syndrome associated with lymph node, thoracic, and pulmonary silicone infiltration following breast implant rupture: experience with four cases.

Silicone has been considered biologically inert; thus it has been employed in many medical devices and nowadays is commonly used in plastic surgery for mammary prosthesis. It is well tolerated in most cases. However, autoimmune disorders and siliconomas with granulomatous reactions after silicone implant rupture have been described. We report cases of four women who developed systemic disorders following rupture of silicone breast implants resulting in lymph node and thoracic silicone infiltration. The symptoms in these cases, including arthralgia, myalgia, generalized weakness, severe fatigue, sleeping disturbances, cognitive impairment, memory loss, irritable bowel syndrome, and weight loss, clearly match the criteria of the recently defined autoimmune/inflammatory syndrome induced by adjuvants (ASIA).

Hyponatraemia in older people as a sign of adrenal insufficiency: a case-control study.

BACKGROUND: Hyponatraemia is a common cause of hospitalisation in older adults. Adrenal insufficiency (AI) can result in hyponatraemia. AIM: The aim of our study was to determine the frequency and characteristics of AI in elderly patients with hyponatraemia. METHODS: Thirty patients ≥65 years with Na(+) ≤130 mmol/L and 30 age-matched control subjects, all hospitalised, were included in the study. Plasma cortisol levels were determined before and after intravenous administration of 1 µg synthetic adrenocorticotropin hormone. A peak cortisol >550 nmol/L was considered to exclude AI. RESULTS: Sodium levels were 125 ± 5 and 139.8 ± 2 mmol/L in the hyponatremic and control groups respectively. Baseline cortisol <550 nmol/L was found in a half of hyponatremic patients. However, stimulated cortisol levels were compatible with AI in only one case (3%) and none of the controls. The mean cortisol levels were significantly higher in hyponatremic compared with control subjects, both in the basal state (585 ± 215 and 381 ± 135 nmol/L, respectively, P < 0.001) and after stimulation (933 ± 254 and 781 ± 160 nmol/L, P < 0.05). However, the incremental increase in cortisol levels after stimulation was similar in the two groups (361 ± 196 and 403 ± 155 nmol/L) CONCLUSIONS: AI is an uncommon cause of hyponatraemia in older age. Based on this small cohort, AI may be present in 3% of elderly patients with hyponatraemia. AI cannot be excluded by baseline cortisol in a significant minority of hyponatremic patients and further testing with adrenocorticotropin hormone stimulation is needed.

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.

Gallium-67 scintigraphy in polymyalgia rheumatica.

A patient with atypical presentation of polymyalgia rheumatica is presented. Her major symptoms were mild weakness, pain on passive movement of the shoulder and hip girdles, with mild tenderness but no joint stiffness. Muscle enzymes were within the normal range. The diagnostic work-up included gallium-67 scintigraphy, which showed intense uptake in both shoulders, with remarkable improvement following steroid treatment. A prospective study may delineate the possible role of this imaging modality in establishing a diagnosis of polymyalgia rheumatica.

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 comparison of the sensitivity of diagnostic criteria for polymyalgia rheumatica.

OBJECTIVE: To compare the performance of the several different diagnostic criteria sets currently in use for polymyalgia rheumatica (PMR). METHODS: 213 patients attending eight rheumatological centres in eight different European countries were studied. All had recently been referred and were considered by the senior investigator at each centre, selected because of their experience in treatment of PMR, to have this condition. By use of a standard international proforma, the requisite diagnostic points in each criteria set were sought. Sensitivity for each criterion from each set was then calculated, as well as the sensitivity of each criteria set as a whole. RESULTS: Of four criteria sets compared, the Bird (1979) criteria performed best with a sensitivity of 99.5%, and the Hunder (1982) criteria second best, with sensitivity of 93.3%. These both performed significantly better than the two other criteria sets, though each of these was admittedly developed for rather specialised reasons. CONCLUSIONS: Although this study compares homogeneity, we suggest the Bird 1979 or Hunder 1982 criteria should be used whenever possible. Studies that have used alternative criteria may have less sensitivity in diagnosis.

EULAR response criteria for polymyalgia rheumatica: results of an initiative of the European Collaborating Polymyalgia Rheumatica Group (subcommittee of ESCISIT).

OBJECTIVE: To develop response criteria for polymyalgia rheumatica (PMR) for monitoring treatment and comparing alternative treatments regimens. METHODS: 76 patients, mean (SD) age 68.7 (7.7) years, were enrolled. Corticosteroids, and non-steroidal anti-inflammatory drugs (NSAIDs) were the only drugs allowed during the observation period. Erythrocyte sedimentation rate (ESR), C reactive protein (CRP), alpha(2) globulin, serum iron, pain, physician's global assessment (PGA), morning stiffness (MST), muscle tenderness (MT), myalgia, and the elevation of upper limbs (EUL) were determined regularly. The daily corticosteroid and NSAID doses as the corticosteroid response time were recorded. To ensure evaluation of an adequate number of patients (n = 57) week 24 was chosen for final analysis. RESULTS: ESR, CRP, alpha(2) globulin, pain, PGA, MST, myalgia, MT, and EUL showed significant improvement (p<0.0001) at week 24 compared with week 0. Multiple regression analysis showed that changes of ESR (p = 0.08), CRP (p = 0.41), alpha(2) globulin (p = 0.13), MST (p = 0.1), and MT (p = 0.07) were independent of pain, but myalgia (p<0.001) and EUL (p = 0.003) were pain dependent. Consequently, a core set of PMR response criteria, comprising ESR or CRP, pain, PGA, MST, and EUL was established. Assessment of treatment responses with this core set resulted in 90%, 70%, 50%, and 20% improvement in 31/57 (54%), 46/57 (81%), 51/57 (89%), and 54/57 (95%) of the patients, respectively. CONCLUSION: These PMR response criteria are a promising tool for better monitoring of disease activity and treatment in PMR. It is proposed that these criteria should be used in clinical trials in the near future to explore alternative treatment options for PMR.

Decreased salivary output in patients with Gaucher disease.

BACKGROUND: Gaucher disease, the most common sphingolipid storage disease, results in accumulation of glucocerebroside in macrophages or "Gaucher cells". In a preliminary screening of 109 patients with type I disease, when asked specifically about dry mouth, approximately one quarter claimed to suffer from this symptom. AIM: To ascertain whether decreased salivary output is a feature of Gaucher disease. DESIGN: Prospective case-control study. METHODS: Salivary output was measured in 65 adult patients and 65 healthy controls using the Saxon test with Hochberg's modification. RESULTS: Mean salivary output was 1.91+/-1.19 g/min in the patient group vs. 2.74+/-1.17 g/min in the control group (p<0.001). This difference was greater among males. These results were not improved in the patients receiving enzyme replacement therapy, which is effective in ameliorating most Gaucher-related signs and symptoms. DISCUSSION: Recent studies have implicated an association between sicca syndrome and viral hepatitis C infection, which may imply an immunological trigger for these findings, but in this specific cohort, only three patients were reactive for hepatitis C. Follow-up of patients, both untreated and receiving enzyme therapy, is needed to delineate the association with salivary hypofunction, and ascertain whether enzyme therapy may induce sicca symptoms.

Charles Bonnet syndrome in glaucoma patients with low vision.

PURPOSE: To characterize the nature and frequency of Charles Bonnet syndrome in glaucoma patients with low vision. PATIENTS AND METHODS: All patients attending the glaucoma clinic during a period of 10 months who had visual acuity of 20/80 or less in both eyes were included in this study. Each patient was questioned about the occurrence of visual hallucinations. Those who responded positively had a thorough interview relating to the characteristics of the hallucinations. Medical history and social history were taken, followed by a complete ocular examination. RESULTS: Eighty-nine patients met the inclusion criteria. Eleven patients (12.3%), eight men and three women, admitted to having experienced visual hallucinations. Except for one case, the patients did not disclose this experience previously. Eight patients had one repeatable hallucination, and three patients experienced more than one sight. The visions were usually sharp, and the figures were occasionally incomplete. Most hallucinations were chromatic. Frequency of hallucinations varied between daily and weekly, and duration was mostly a few minutes. In addition to glaucoma, nine of the eleven patients had other ocular findings that could have contributed to the reduction of vision. CONCLUSION: Visual hallucinations are not rare in glaucoma patients with low vision. Patients tend to conceal their experience of visual hallucinations, but a discussion of these phenomena with the patient and assurance of their harmless nature will reduce his or her anxiety and concerns.

Visual hallucinations in giant cell arteritis: association with visual loss.

OBJECTIVE: To evaluate the frequency and characteristics of visual hallucinations (VH) in patients with giant cell arteritis (GCA) and to determine their relationship to other visual phenomena. METHODS: This prospective study included 31 consecutive patients with GCA. All were asked whether they had experienced recent visual phenomena. Patients with visual symptoms underwent a comprehensive ophthalmologic examination. When unusual visual phenomena were reported, patients were asked to describe their nature, duration, and frequency of occurrence. RESULTS: Visual symptoms occurred in 6 patients: permanent visual loss in 5 and amaurosis fugax in one. In 4 of the 5 patients with permanent visual loss, it was preceded by intermittent VH over a period of 1-10 days. Patients were aware of the unreal nature of the visions. Hallucinations disappeared within 2 weeks, but in one patient, recurred 6 months later in association with further visual deterioration. CONCLUSION: The occurrence of visual hallucinations in patients with GCA-associated visual loss is more common than previously appreciated. As hallucinations preceded permanent loss of vision, this phenomenon may serve as a harbinger of imminent visual loss.

Anti-nuclear envelope antibodies: Clinical associations.

OBJECTIVES: Characterization of the clinical associations and clinical implications of antibodies reacting with antigens of the nuclear envelope. METHODS: Description of an illustrative case and a MEDLINE search-assisted literature review of relevant cases. RESULTS: With indirect immunofluorescence, autoantibodies directed against various antigens of the nuclear envelope stain the nucleus in a ring-like (rim) pattern. Autoantibodies against 5 antigenic components of the nuclear envelope have been described: anti-gp210, p62, lamina, lamina-associated polypeptides, and lamin B receptor. Antibodies to antigens of the nuclear pore complex, such as gp210 and p62, are highly specific (> 95%) for primary biliary cirrhosis and may aid in the serologic diagnosis of this condition, especially in cases in which antimitochondrial antibodies are not detectable. In contrast, antilamin antibodies are not disease-specific but seem to be associated with lupus anticoagulant or anticardiolipin antibodies, antiphospholipid syndrome, thrombocytopenia, autoimmune liver diseases, and arthralgia. High-titered antilamin antibodies help to define a subset of lupus patients with antiphospholipid antibodies who are at a lower risk of developing thrombotic events. In addition, preliminary data suggest that the presence of antilamin antibodies may be helpful in the diagnosis of chronic fatigue syndrome. CONCLUSIONS: Each of the antibodies reacting with nuclear membrane antigens has its own spectrum of disease associations. RELEVANCE: Determination of anti-nuclear envelope antibody pattern by indirect immunofluorescence, with subsequent determination of the specific antibody, carries important diagnostic and prognostic implications in various autoimmune conditions.

Neurologic manifestations of giant cell arteritis.

Neurological manifestations are common in giant cell arteritis. Both the central and peripheral nervous system can be involved. The most dreaded manifestations are visual loss and stroke. Both frequently have premonitory symptoms, such as amaurosis fugax, blurry vision, diplopia, transient ischemic attacks, and jaw claudication. Although most of these manifestations occur prior to steroid therapy, they may also develop during the early phase of therapy, or during tapering of the dose of steroids. Earlier diagnosis, close monitoring and improving the treatment protocols may prevent mortality and improve morbidity in these cases.

Palindromic rheumatism: effect of dietary manipulation.

OBJECTIVE: Evaluation of the contribution of dietary components in triggering the attacks of palindromic rheumatism (PR), and the effect of dietary manipulation on the frequency and severity of PR attacks. METHODS: Sixteen patients (10 males, 6 females) were diagnosed as having PR during 1994-8 in one center. Their mean age was 45 +/- 6, duration of symptoms prior to diagnosis was 4 +/- 1.4 years, and frequency of PR attacks were 3.1 +/- 1.8/month. All patients were instructed to make a list of the food that was consumed daily and to specify the dates of PR episodes. Data were evaluated after a period of 2-4 months in each patient. RESULTS: In 5 patients (31%) there was an association between episodes of PR and certain foods that were consumed within 36 hours prior to PR episodes. These were fish (2 patients), eggs, canned vegetables and processed cheese (each in one case). Elimination of the relevant food from each patient's diet resulted in complete cessation of the PR attacks in two of the cases, while the other three had milder, infrequent attacks. Four patients were rechallenged with the offending food. In all cases it resulted in recurrence of the PR attacks. No association between PR episodes and prior consumption of certain foods could be documented in the other 11 patients. CONCLUSIONS: In some PR patients ingestion of certain foods, specific for each case, can trigger the typical attack. It is suggested that this association should be looked for in any PR patient, as elimination of the offending food from the diet may help in preventing the PR attacks.