Systemic lupus erythematosus in northwestern Spain: a 20-year epidemiologic study

To further investigate the epidemiology of systemic lupus erythematosus (SLE) in southern Europe, we assessed the incidence, prevalence, clinical spectrum of the disease, flares, and survival of patients diagnosed with SLE in the Lugo region of northwestern Spain. Between January 1987 and December 2006, 150 Lugo residents were diagnosed as having SLE according to the 1982 American College of Rheumatology criteria for the classification of SLE. Women outnumbered men (127 [84.7%] vs. 23 [15.3%]). The mean age at the time of disease diagnosis was 46.1 ± 19.6 years. The mean follow-up from the time of disease diagnosis was 7.8 ± 4.5 years. The age- and sex-adjusted annual incidence rate over the 20-year study period was 3.6 (95% confidence interval [CI], 3.0-4.2) per 100,000 population aged 15 years and older. The overall annual incidence rate over the 20-year study period in women (5.9/100,000 population aged ≥ 15 yr; 95% CI, 4.9-7.0) was higher than in men (1.1/100,000 population aged ≥ 15 yr; 95% CI, 0.7-1.7) (p < 0.001). By December 31, 2006, the overall age-adjusted SLE prevalence in the Lugo region for patients who fulfilled at least 4 of 1982 American College of Rheumatology criteria was 17.5 per 100,000 population aged 15 years and older (95% CI, 12.6-24.1). Prevalence in women (29.2/100,000 population aged ≥ 15 yr; 95% CI, 20.0-40.7) was higher than in men (5.8/100,000 population aged ≥ 15 yr; 95% CI, 2.0-12.0). The most frequent clinical manifestation was arthritis. As reported in population-based studies on SLE patients of European descent, renal disease was observed in only 27.3% of the patients. The rate of flares was 0.084/year. A younger age and the presence of nephritis at the time of disease diagnosis were associated with the development of flares during the follow-up of Lugo patients. Compared with the general population the probability of survival in patients with SLE was significantly reduced (p = 0.04). In conclusion, the present study establishes a baseline estimate of the incidence and clinical spectrum of SLE in northwestern Spain. According to our results, the incidence of SLE in northwestern Spain is slightly higher than that reported in most European regions. Patients with SLE from northwestern Spain have a later average age onset and a lower frequency of nephritis than in the African-American population. However, our data show a reduced probability of survival in Spanish patients with SLE.     

Giant cell arteritis in northwestern Spain: a 25-year epidemiologic study

To continue our investigation of the epidemiology of giant cell arteritis (GCA) in southern Europe, we assessed the potential presence of trends, peaks, and fluctuations in the incidence of this vasculitis over a 25-year period in the Lugo region of northwestern Spain. We also sought to determine whether changes in the clinical spectrum of the disease existed. From 1981 to 2005, biopsy-proven GCA was diagnosed in 255 Lugo residents. The age- and sex-adjusted annual incidence rate was 10.13 (95% confidence interval [CI], 8.93-11.46) per 100,000 population aged 50 years and older. The mean age +/- SD at the time of diagnosis was 75.0 +/- 6.9 years. The annual incidence rate in women (10.23; 95% CI, 8.60-12.08) was slightly greater than that in men (9.92; 95% CI, 8.19-11.89) (p = 0.15). The annual incidence rate increased with advancing age up to a maximum of 23.16 (95% CI, 19.52-27.28) in the 70-79 year age-group. A progressive increase in the incidence was observed from 1981 through 2000 (p = 0.001). However, the age- and sex-adjusted incidence rate for biopsy-proven GCA in the Lugo region did not show peaks in the annual incidence of GCA. Likewise, we observed no seasonal pattern for the diagnosis of the disease. Visual ischemic manifestations and irreversible visual loss were observed in 57 (22.4%) and 32 (12.5%) of the 255 patients, respectively. A negative trend manifested by a progressive decline in the number of patients with visual ischemic manifestations (p = 0.021) or permanent visual loss (p = 0.018) was found over the 25-year period of study. The decline in the frequency of visual manifestations of GCA could not be attributed to a shorter delay to diagnosis, as no significant differences were observed when the delays to diagnosis in the 5 consecutive 5-year periods were compared. In conclusion, the current study confirms a progressive increase in the incidence of biopsy-proven GCA in northwestern Spain, and suggests that there has been a change in the clinical spectrum of the disease.     

Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects.

The vasculitides constitute a heterogeneous group of diseases characterized by blood vessel inflammation and necrosis with different but frequently overlapping clinical and pathologic manifestations. The incidence of these conditions is frequently controversial. To further investigate the incidence and clinical manifestations of vasculitides, we reviewed the spectrum of these diseases in an unselected population of adults (age > 20 years) from northwestern Spain during a 10-year period. From January 1988 through December 1997, 267 adults were diagnosed as having vasculitis. The overall average annual incidence rate of vasculitis in the region of Lugo, Spain, between 1988 and 1997 for the population older than 20 years was 141.54/million. Primary vasculitis (115.04/million for the population older than 20 years; 81.3%), especially giant cell arteritis (GCA) was the most common group. Small vessel primary vasculitis (hypersensitivity vasculitis and Henoch-Sch?nlein purpura) was the second most common group. Both GCA and small vessel primary vasculitis had a good outcome. However, although less common, patients with medium and small vessel primary vasculitis, in particular those with polyarteritis nodosa, had a high mortality related to the systemic manifestations of the disease or to the immunosuppressive therapy. Among the group of adults with secondary vasculitis (26.51/million; 18.7%), rheumatic diseases and specifically those occurring in the context of rheumatoid arthritis were the most common group. Patients with secondary vasculitis had clinical or laboratory data that may suggest the presence of an underlying disease. In summary, systemic vasculitides are somewhat more common than previously considered. As in other western countries, GCA constitutes the most common type of vasculitis in northwestern Spain. Better physician awareness may contribute to the progressive increase in the recognition of these conditions.     

Polymyalgia rheumatica: a 10-year epidemiologic and clinical study

Ninety-six patients with polymyalgia rheumatica were identified in Olmsted County, Minnesota, during the 10-year period 1970 to 1979. Giant cell arteritis was found in 15 of the 96 patients. The average annual incidence of polymyalgia rheumatica in the population increased from 19.8 per 100 000 in persons 50 to 59 years of age, to a maximum of 112.2 per 100 000 in persons 70 to 79 years of age. Eighty-three of the 96 patients (86%) had recovered by the end of the study. Median duration of the disease was 11 months (range, 2 to 54 months). Polymyalgia rheumatica had no effect on survival. Both corticosteroids and nonsteroidal anti-inflammatory drugs were used in treatment. Response was more rapid in patients given corticosteroids, but nonsteroidal drugs were used successfully, especially in milder cases. Relapses and adverse reactions to treatment were more frequent in patients given corticosteroids. The findings show that polymyalgia rheumatica is a relatively common disease in middle-aged and older persons and generally runs a self-limited course.     

Systemic lupus erythematosus in Martinique: an epidemiologic study

PURPOSE: To review epidemiological and clinical aspects of systemic lupus erythematosus (SLE) in Martinique, French West Indies. METHODS: Cases of SLE were identified by attending physicians. Patients who presented with at least four of the criteria defined by the American College of Rheumatology were included. Determination of incidence and prevalence included the new cases arising during the 1990-1999 period and 1999 population census results. Probability of survival was based on the use of the Kaplan-Meier estimator. RESULTS: Two hundred and eighty-six patients were studied, including 265 females (92.7%). The average annual incidence was 4.7 cases per 100,000 inhabitants (95% confidence interval [CI]: 2.5-6.9). The prevalence for 1999 was 64.2 cases per 100,000 inhabitants (CI: 56.2-72.2). The mean age at onset was 30 years. Eleven percent of all patients had at least one parent with SLE. Renal disease was present in 139 patients (48.6%), and neurological disorders were diagnosed in 70 patients (24.5%). Patients tested positive for the following antibodies: anti-Sm (37.1%), anti-RNP (58.7%), anti-SSA (47.2%). Mean survival time was: 96.4% (CI: 94.1-98.7) at 5 years, 91.8% (CI: 87.9-95.7) at 10 years. Survival was significantly reduced in patients with end-stage renal disease (n = 40, chi 2 = 6.96, P < 0.01). CONCLUSION: The high incidence of SLE in Martinique and the immunological characteristics of patients were found to be similar to those described in other populations of African descent. The frequency of renal disease and survival rates were identical to those reported in Caucasians.     

Human trichinellosis in Sourthern Spain: serologic and epidemiologic study

An outbreak of trichinellosis caused by wild boar meat occurred in the Iruela (Jaen) in southern Spain in February 1996. Thirty-five people were diagnosed on the basis of epidemiologic data, but only 24 patients agreed to participate in this study. Twenty-three (96%) had symptoms suggestive of trichinellosis. Immunofluorescent and Western blot test results for trichinellosis were positive in 18 persons, and 15 had circulating Trichinella spiralis antigens. These findings suggest that results of tests for circulating antigens in conjunction with clinical presentation are useful for the diagnosis of trichinellosis.     

Variceal sclerosis in schistosomotic patients: a 5-year follow-up study

To assess the therapeutic possibilities of injection sclerosis in schistosomotic portal hypertension, a 5-year prospective study was conducted in northeast Brazil, where this parasitosis is endemic. Fifty patients undergoing endoscopy for upper gastrointestinal hemorrhage from rupture of esophageal varices from July through December 1981 were chosen for the study. The 32 consenting patients were submitted to injection sclerotherapy paravariceally, using ethanolamine oleate; the 18 refusing to participate were assigned to the control group. The incidence of rebleeding was 28.1% in the former and 44.5% in the latter, a difference which was not statistically significant (Fisher's test, p = 0.375). Mortality from rupture of esophageal varices was 3.1% in the sclerotherapy group and 27.7% in the control group, a statistically significant difference (Fischer's test, p = 0.017). Since sclerotherapy markedly improved the long-term survival rate of the patients, this procedure is advocated for the treatment of esophageal varices in cases of portal hypertension due to schistosomiasis.     

An epidemiologic study of acute coronary syndromes in northwestern Greece

Acute coronary syndromes (ACS) represent the most common cause of morbidity and mortality in the Western world. Relative epidemiologic data for Greece, a Mediterranean country, are sparse. The aim of the study was to determine the incidence and the clinical presentations of ACS. Over a 1-year period we conducted a prospective, population-based survey of ACS cases in an isolated area of northwestern (NW) Greece with 170,000 inhabitants. Every patient living in the study area, aged <80 years, without history of coronary artery disease, who presented with symptoms suspicious for ACS and was hospitalized for at least 24 hours was eligible for inclusion in the study. For sudden cardiac deaths, relative information was obtained from the autopsy report or the physician who documented death. Additional information regarding timing and associated conditions was obtained from relatives. The diagnosis and classification of the studied cases was performed according to World Health Organization and European Society of Cardiology criteria. The authors identified 352 patients (265 men, 87 women, mean age 62.5 +/-10 and 68 +/-9.5 years, respectively) with first-appeared ACS (174 non-ST elevation, 105 ST elevation, 73 sudden cardiac deaths). Fifty-six patients with other forms of ischemic heart disease (stable angina, heart failure, and silent ischemia) were not included in the analysis. Moreover, 154 patients with chest pain and normal appearing EGG at rest, normal values for enzymes (CK, troponin), and negative exercise testing, as well as 77 patients with normal findings from coronary angiography studies, were also excluded. The annual incidence for the age group of 30-79 was 39/10,000 inhabitants (60.6 for men and 19 for women). The incidence of ACS increased with age in both sexes and was higher in men even after the age of 70. About one third of the ACS and half of the sudden cardiac deaths occurred in the age group of 70-79. Only 3 patients were successfully resuscitated. ACS are common in this area of NW Greece and the majority of them present in a form amenable to therapeutic manipulations. Twenty percent of patients died suddenly, and a very small percentage of these were successfully resuscitated. Preventive measures and acute management facilities need to be improved, even in a Mediterranean country where the incidence of ischemic heart disease is relatively lower than in northern European countries.     

Temporal arteritis: a 25-year epidemiologic, clinical, and pathologic study

Among the population of Olmsted County, Minnesota, 42 patients with temporal arteritis were identified during a 25-year period. The average annual incidence per 100 000 population aged 50 and older rose from 5.1 in 1950-1959 to 17.4 in 1970-1974. The prevalence of patients with a history of the diagnosis of temporal arteritis on 1 January 1975 was 133 per 100 000 population aged 50 and older. All patients received corticosteroid therapy for a range of 1 to 77 months (median, 7 months). Relapses in 10 of 11 patients were associated with corticosteroid reduction. The majority of patients recovered fully and were followed off corticosteroids for 10 months to 19 years (median, 5 years). Temporal arteritis had no significant effect on survival. Vertebral compression fractures and myopathy were the most serious complications of therapy. The presence of giant cells in biopsies was in part related to the number of sections examined, and their presence had no apparent influence on the clinical course.     

The clinical spectrum of severe septic bursitis in northwestern Spain: a 10 year study

OBJECTIVE: To assess the clinical and microbiological characteristics of septic bursitis in those cases that required treatment at the hospital during the past 10 years in a northwestern area of Spain. METHODS: The charts of all patients diagnosed as having septic bursitis at Hospital Xeral-Calde, Lugo, Spain, from October 1987 through September 1997 were reviewed based on published criteria and graded according to severity. RESULTS: Sixty-nine patients diagnosed with definite and 6 with probable septic bursitis met the criteria for severe septic bursitis. Sixty-two were male (82.7%). The mean age at the time of diagnosis was 51 years. The most frequently involved sites were olecranon (47%) and prepatellar (44%) bursae. Among predisposing factors, the presence of prepatellar bursitis was correlated with a job that involved frequent trauma on the bursae. The main clinical and laboratory findings were cellulitis and/or erythema (94.7%), fever (77.3%), and leukocytosis (72%). Noninflammatory synovial fluid (SF, < 2,000 leukocytes/mm3) was observed in 4/32 (12.5%) cases. Positive SF cultures were obtained in 69 of 75 patients (92%). Staphylococcus aureus was the most common pathogen (84%). Blood cultures were positive in 12 of 62 patients (19.4%). Three patients had osteomyelitis. This complication was associated with a longer delay to diagnosis from the onset of symptoms (> 3 weeks vs 9.3+/-13.3 days for the group as a whole). Apart from these 3 cases, overall outcome was excellent. CONCLUSION: Severe septic bursitis is a common disease. Local trauma is the most common risk factor for this infection. Although the most common pathogen is S. aureus, other pathogens such as Brucella abortus play an important role in this infection in our area.     

Cancer and systemic sclerosis. An epidemiologic study

The association between systemic sclerosis and malignancy was evaluated in the Pittsburgh standard metropolitan statistical area during 1971-1982 and compared with data for this geographic area from the Third National Cancer Survey of 1969-1971. Fourteen malignancies were detected in 262 systemic sclerosis patients (5%) during a followup period that included 1,335 patient-years. After adjustment for age and sex, the expected number of malignancies was 7.72 (relative difference = 1.81; P = 0.05). This increased relative difference was predominantly due to an increase in observed lung cancer (relative difference = 4.4; P less than 0.05), which occurred in the setting of long-standing pulmonary fibrosis but was not associated with cigarette smoking. Although breast cancer was no more frequent than expected, it tended to occur in close temporal relationship with the onset of systemic sclerosis. These findings suggest a biologic relationship between systemic sclerosis and certain malignant neoplasms.     

Temporal arteritis in a northwestern area of Spain: study of 57 biopsy proven patients.

Fifty-seven patients, diagnosed with temporal arteritis by biopsy from 1981 to 1990, were studied. The average annual incidence rate/100,000 population aged 50 and older, which was slightly lower than those from other Mediterranean countries of Europe, was 6. Apart from a predominance of males, age, clinical and laboratory features were similar to those reported from other parts of the world. All patients received corticosteroid therapy, the majority of them recovered completely. Fifty-four were followed; 34 had already finished treatment (mean: 22 months, range: 12-50). Relapses occurred more commonly after 12 months of therapy, when the amount of prednisone given was low or discontinued.     

Luberculous spondylitis: epidemiologic and clinical study in non-HIV patients from northwest Spain

OBJECTIVES: To study the epidemiology, clinical features, and outcome of non-human immunodeficiency virus (HIV) patients diagnosed with tuberculous spondylitis (TS) in a well-defined region of northwestern Spain. METHODS: Retrospective chart review of patients older than 14 years of age diagnosed with TS at two contiguous areas between 1986 and 1999. RESULTS: Thirty-seven patients (19 men; mean age 60.3 years) were diagnosed with TS. The average annual incidence rate of TS was 0.55/100,000 population 15 years of age and older. The thoracic and lumbar regions were affected in most cases. The mean duration of symptoms before diagnosis was 28 weeks (range 3-129). Active or healed pulmonary tuberculosis was observed in only 30%. The tuberculin skin test was negative in 24%. The most common findings at the time of diagnosis were back pain and elevated ESR (either 89%). Of note, only 19% had fever. On admission plain radiographs disclosed the presence of spondylitis in 84% of the patients. Computed tomography scan and magnetic resonance imaging yielded conclusive diagnostic data in the cases with normal radiographs, and were very useful in the visualization of abscesses and intraspinal compression. Cultures of material from percutaneous needle aspiration and open bone biopsy were positive for Mycobacterium tuberculosis in 79% and 77% of the cases, respectively. Antituberculous therapy was given to all patients (mean duration of treatment 44 weeks). Surgical procedures were performed in 12 cases, in 7 of them to remove paraspinal and/or epidural abscesses, and in 5 because of neurological complications. Local pain and neurological deficits were the mostfrequent sequelae (16 and 8 cases, respectively). One patient died during the course of treatment due to a co-morbid disease. None of the patients had relapses of tuberculosis. CONCLUSION: TS is a major cause of morbidity. There is a long delay to the diagnosis in most patients. Awareness of its clinical features and early therapy are required to reduce severe complications.     

Systemic Autoimmune Diseases in Patients Hospitalized with COVID-19 in Spain: A Nation-Wide Registry Study

We aimed to evaluate the clinical outcome of Systemic Autoimmune Diseases (SADs) patients hospitalized with COVID-19 in Spain, before the introduction of SARS-CoV-2 vaccines. A nationwide, retrospective and observational analysis of the patients admitted during 2020, based on the ICD10 codes in the National Registry of Hospital Discharges, was performed. Among 117,694 patients, only 892 (0.8%) presented any type of SAD before COVID-19-related admission: Sjogren’s Syndrome constituted 25%, Systemic Vasculitides 21%, Systemic Lupus Erythematosus 19%, Sarcoidosis 17%, Systemic Sclerosis 11%, Mixed and Undifferentiated Connective Tissue Disease 4%, Behçet’s Disease 4% and Inflammatory Myopathies 2%. The in-hospital mortality rate was higher in SAD individuals (20% vs. 16%, p < 0.001). After adjustment by baseline conditions, SADs were not associated with a higher mortality risk (OR = 0.93, 95% CI 0.78–1.11). Mortality in the SADs patients was determined by age (OR = 1.05, 95% CI 1.04–1.07), heart failure (OR = 1.67, 95% CI 1.10–2.49), chronic kidney disease (OR = 1.29, 95% CI 1.05–1.59) and liver disease (OR = 1.97, 95% CI 1.13–3.44). In conclusion, the higher COVID-19 mortality rate seen in SADs patients hospitalized in Spain in 2020 was related to the higher burden of comorbidities, secondary to direct organ damage and sequelae of their condition. Whilst further studies should evaluate the impact of baseline immunosuppression on COVID-19 outcomes in this population, efforts should be focused on the optimal management of SAD to minimize the impact of the organ damage that has been shown to determine COVID-19 prognosis.     

Systemic sclerosis is associated with knee arthroplasty outcomes: a National US study

Clinical Rheumatology - To assess whether systemic sclerosis (SSc) is associated with total knee arthroplasty (TKA) outcomes. We used the 1998–2014 US National Inpatient Sample. We conducted...     

Systemic sclerosis in two second cousins: a case report

We describe two second cousins who developed systemic sclerosis. These patients had major histocompatibility complex (MHC) class I alleles in common, including A2, A26(10), B60(40), and Cw7 as well as class II allele DR2. This DR2 was thought to be associated with the onset of the disease. Our patients both experienced a limited type of systemic sclerosis, but the expression of autoantibodies was different. Received: September 20, 2000 / Accepted: January 9, 2001     

Aortic aneurysm and dissection in patients with biopsy-proven giant cell arteritis from northwestern Spain: a population-based study

Most classical manifestations of giant cell arteritis (GCA) are the result of occlusive vascular involvement. However, unlike ischemic manifestations, aortic aneurysmal disease in patients with GCA has been less well described. We assessed the incidence and predictors of aortic aneurysm and dissection in patients with biopsy-proven GCA from the Lugo region of northwestern Spain and compared the results with those in a 2003 report from Olmsted County, MN. We performed a retrospective study of biopsy-proven GCA patients diagnosed from 1981 to 2001 at the single hospital for a well-defined population of almost 250,000 people. Twenty (9.5%) of the 210 biopsy-proven GCA patients diagnosed during the study period developed aortic aneurysmal disease. Sixteen of the 20 patients had thoracic aneurysms and 6 had abdominal aneurysms. The incidence of aortic aneurysm and/or dissection in Lugo (18.9 per 1000 person years at risk) was similar to that reported in Olmsted County (18.7 per 1000 person years at risk). Hypertension (hazard ratio: 4.73) and polymyalgia rheumatica with a marked acute inflammatory response at the time of diagnosis of GCA (hazard ratio: 3.71) were the best predictors of aortic aneurysmal disease. Our present observations suggest that a severe inflammatory response associated with hypertension at the time of diagnosis of GCA may promote the development of aortic aneurysmal disease. GCA patients having these features should be monitored for the existence of aortic aneurysm and dissection.