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Biomedical subjects

J Mañá

Publications and source records attributed to J Mañá.

At least 19 recordsLinked to original sources
PubMed2005

Association between ACE gene I/D polymorphism and clinical presentation and prognosis of sarcoidosis.

Serum angiotensin converting enzyme (SACE) concentration is considered a marker of sarcoidosis activity. This concentration is influenced by an insertion/deletion (I/D) polymorphism of the ACE gene, such that SACE levels follow the pattern DD>ID>II. The aim of our work was to study the relationship between I/D polymorphism and susceptibility to sarcoidosis, as well as the relation between this polymorphism and the clinical presentation and evolution of the disease in 177 sarcoidosis patients. A group of 104 individuals without sarcoidosis was included as control. Genotyping was done by a polymerase chain reaction (PCR) method, and SACE concentration at diagnosis was determined by a kinetic method. No differences were observed in genotype or allele distributions between patients and controls, nor between patients considering the type of presentation (Löfgren versus non-Löfgren) and evolution of the disease (acute versus chronic). As reported for healthy populations, SACE concentrations followed the pattern DD>ID>II in sarcoidosis patients, but significant differences between genotypes existed only in the Löfgren group (p = 0.003) and in acute patients (p = 0.02). SACE concentrations at diagnosis were lower in acute patients (p = 0.05) and in Löfgren's syndrome (p = 0.04), but this seemed to occur only in ID individuals (p = 0.02 and p = 0.01, respectively). No relation was thus found between I/D polymorphism and susceptibility to sarcoidosis, but ACE I/D genotyping may improve the assessment of disease activity, both at diagnosis and during the follow-up of treated and untreated patients.

Female↗
PubMed2005

Systemic autoimmune diseases co-existing with chronic hepatitis C virus infection (the HISPAMEC Registry): patterns of clinical and immunological expression in 180 cases.

OBJECTIVES: To describe the clinical and immunologic characteristics of a large series of patients with systemic autoimmune diseases (SAD) associated with chronic hepatitis C virus (HCV) infection. METHODS: We analysed 180 patients diagnosed with SAD and chronic HCV infection seen consecutively at our centres during the last 10 years. The clinical and immunological patterns of disease expression were compared with 180 SAD-matched patients without chronic HCV infection. RESULTS: A total of 180 HCV patients fulfilled the classification criteria for the following SAD: Sjogren's syndrome (n = 77), systemic lupus erythematosus (n = 43), rheumatoid arthritis (n = 14), antiphospholipid syndrome (n = 14), polyarteritis nodosa (n = 8) and other SAD (n = 24). One hundred and thirty (72%) patients were female and 50 (28%) male, with a mean age at SAD diagnosis of 50 years. The main immunologic features were antinuclear antibodies in 69% of patients, cryoglobulinaemia in 62%, hypocomplementaemia in 56% and rheumatoid factor (RF) in 56%. Compared with the SAD-matched HCV-negative group, SAD-HCV patients presented a lower prevalence of females (P = 0.016), an older age at SAD diagnosis (P = 0.039) and a higher prevalence of vasculitis (P < 0.001) and neoplasia (P < 0.001). Immunologically, SAD-HCV patients presented a lower prevalence of antinuclear (P = 0.036), anti-extractable nuclear antigen (P = 0.038) and anti-DNA (P = 0.005) antibodies, and a higher frequency of RF (P = 0.003), hypocomplementaemia (P < 0.001) and cryoglobulins (P < 0.001). CONCLUSIONS: In comparison with an SAD-matched HCV-negative population, SAD-HCV patients were older and more likely to be male, with a higher frequency of vasculitis, cryoglobulinaemia and neoplasia. This complex pattern of disease expression is generated by a chronic viral infection that induces both liver and autoimmune disease.

Antibodies, Antinuclear↗
PubMed2001

[Sarcoidosis].

Explore the source record for details and available documents.

Administration, Topical↗
PubMed2001

Serum amyloid A and high-density lipoprotein cholesterol: serum markers of inflammation in sarcoidosis and other systemic disorders.

Hypocholesterolemia has been observed in several inflammatory diseases such as rheumatoid arthritis, myeloproliferative disorders, systemic lupus erythematosus and sarcoidosis. Serum amyloid A is an acute-phase reactant that is related to the high-density lipoprotein cholesterol. This review discusses the relationship between the activation of the cells of the monocyte-macrophage system, determined by the serum amyloid A levels, and the lipid metabolism, measured as alterations in plasma lipoprotein concentrations. The mechanisms of this association during acute inflammation are also discussed in this review.

Apolipoproteins↗
PubMed2001

Foreign bodies in granulomatous cutaneous lesions of patients with systemic sarcoidosis.

OBJECTIVE: To assess the presence of foreign material in the granulomatous cutaneous lesions of patients with systemic sarcoidosis. DESIGN AND SETTING: Observational study reevaluating histological specimens at a university referral hospital. PATIENTS: Sixty-five patients diagnosed as having sarcoidosis who developed granulomatous cutaneous involvement. MAIN OUTCOME MEASURES: To detect the presence of polarizable foreign particles in cutaneous biopsy specimens and to evaluate the association with clinical features of the patients. RESULTS: Granulomatous cutaneous involvement was demonstrated in 65 (15.3%) of 425 patients with systemic sarcoidosis. In 14 (22%) of the 65 patients, the cutaneous biopsy specimen showed foreign particles in polarized light. The skin lesions corresponded to 3 different clinical patterns: an admixture of papules and infiltration of previously undetected minute scars (n = 6); scar sarcoidosis (n = 4); and subcutaneous nodules (n = 4). The lesions were located most frequently in the extremities, involving the knees in 10 patients. CONCLUSIONS: The presence of polarizable foreign body material in granulomatous cutaneous lesions is not infrequent in patients with systemic sarcoidosis. Inoculation of foreign matter from a previous inapparent minor trauma may induce granuloma formation in individuals with sarcoidosis.

Adult↗
PubMed1999

Löfgren's syndrome revisited: a study of 186 patients.

PURPOSE: To evaluate the clinical features, the results of noninvasive tests and biopsies, and the outcome of patients with Löfgren's syndrome. SUBJECTS AND METHODS: Patients diagnosed as having Löfgren's syndrome at a university hospital in Barcelona, Spain, from 1974 to 1996, were prospectively followed. Löfgren's syndrome was defined as the association of erythema nodosum or periarticular ankle inflammation with unilateral or bilateral hilar or right paratracheal lymphadenopathy. RESULTS: Löfgren's syndrome was diagnosed in 186 patients. The mean age was 37 +/- 11 years, and 157 (85%) were women. In 91 patients (49%), symptoms started during the spring (P < 0.0001). Erythema nodosum, periarticular ankle inflammation, or both were present at onset in 173 patients (93%). At the time of diagnosis, 161 patients (87%) had no respiratory symptoms; 151 (81%) had stage I abnormalities on chest radiograph, 29 (16%) stage II, and 6 (3%) stage 0. Five percent of patients had decreased forced vital capacity, and 15% had decreased carbon monoxide diffusing capacity. Extrathoracic involvement was infrequent. Serum angiotensin-converting enzyme levels were increased in 50% of patients. Gallium-67 scans showed hilar uptake in all the studied patients, but it yielded useful additional diagnostic information only in those with normal chest radiographs or with unilateral hilar lymphadenopathy. The diagnosis was proven with biopsy results in 63% of patients. None of the patients without histologic confirmation were subsequently found to have a diagnosis other than sarcoidosis. In the 133 patients who were followed for a mean of almost 5 years, 11 (8%) continued to have active disease, and 8 (6%) had several recurrences between 18 months and 20 years after a complete resolution. A normal serum angiotensin-converting enzyme level at diagnosis was associated with disease resolution without recurrence. CONCLUSION: Löfgren's syndrome is usually a self-limiting form of sarcoidosis. Histologic confirmation is not necessary in typical cases. In a small number of patients, the disease may remain active or recur long after its onset, although usually with mild organ dysfunction.

Adolescent↗
PubMed1997

Cutaneous involvement in sarcoidosis. Relationship to systemic disease.

Sarcoidosis is an antigen-mediated disease defined by granuloma formation in different organs. It involves mainly the mediastinal and peripheral lymph nodes, lungs, eyes, skin, liver, and spleen. Cutaneous lesions of sarcoidosis may be specific, showing histologically noncaseating granulomas, or nonspecific, most typically erythema nodosum. Frequently, both types of skin lesions are the means of presentation of the disease and may contribute to the diagnosis. A workup for systemic sarcoidosis should be undertaken in every patient with sarcoid cutaneous granulomas. Some types of cutaneous lesions have prognostic significance. Lupus pernio and plaques are associated with more severe systemic involvement and more chronic course, while erythema nodosum is the hallmark of acute and benign disease.

Acute Disease↗
PubMed1997

Nuclear imaging. 67Gallium, 201thallium, 18F-labeled fluoro-2-deoxy-D-glucose positron emission tomography.

67Gallium scan has been used for years in sarcoidosis as a marker of activity, a determiner of the extent and distribution of the disease, a diagnostic support, and an aid in therapeutic management. Because of its limited sensitivity and specificity for sarcoidosis, however, it is currently used mainly to assist in diagnosis in difficult cases, particularly in those with isolated extrathoracic sarcoidosis. The finding of the typical lambda or panda patterns supports the diagnosis and reinforces the indication to perform an appropriate biopsy or Kveim-Siltzbach test. In addition, the detection of clinically silent extrathoracic uptake may provide sites for biopsy. 67Gallium scans' routine use in the follow-up of pulmonary sarcoidosis under treatment has decreased because that is best accomplished by means of serial chest radiographs and PFT. 201Thallium scintigraphy studies the myocardial perfusion and is complementary to echocardiography and 24-hour electrocardiographic monitoring in the assessment of sarcoid cardiac involvement. It typically shows segmental areas of decreased uptake in the ventricular myocardium that disappear or decrease in size during stress or after intravenous administration of dipyridamole. That reverse distribution is not specific for cardiac sarcoidosis, however, because it may also occur in other cardiomyopathies. PET is based on the increase of glucose metabolism in inflamed tissues. It may have great potential to assess sarcoidosis activity, but it is still largely experimental and is not routinely employed.

Fluorodeoxyglucose F18↗
PubMed1996

Are the pulmonary function tests and the markers of activity helpful to establish the prognosis of sarcoidosis?

The prognosis of sarcoidosis is difficult to establish. It depends mainly on the persistence of activity over time and the degree of functional impairment of the involved organs. The aim of this study was to analyze whether the pulmonary function tests and the more commonly used markers of activity, such as serum angiotensin-converting enzyme (SACE) and gallium-67 scan, are helpful to evaluate the prognosis of sarcoidosis, besides the clinical data. Over a 14-year period (1974-1987), 209 patients were diagnosed as having sarcoidosis at the Bellvitge Hospital, a 1,000-bed teaching hospital in Barcelona, Spain. Clinical, radiological, pulmonary function tests, and activity markers (SACE and gallium-67 scan) data at diagnosis were collected and classified as variables, and a definition of disease activity was established. One hundred sixteen patients were on follow-up, had all the variables available and were included in the statistical analysis. A Cox proportional-hazard regression model identified the following variables as independently influencing the persistence of activity over time: absence of erythema nodosum [risk ratio (RR) = 2.78; 95% confidence interval (CI): 1.48-5.18], age > or = 40 years (RR = 1.67; 95% CI: 1.008-1.04), SACE level > or = mean + 2 SD U/ml (RR = 1.45; CI: 0.99-1.07), hyperglobulinemia (RR = 2.47; CI: 0.98-6.24), forced vital capacity < 80% predicted (RR = 2.17; CI: 0.97-4.85), and male sex (RR = 1.8; CI: 0.95-3.45). We conclude that the pulmonary function tests and the SACE level but not the gallium scan are helpful to identify the factors predicting persistence of activity in sarcoidosis. Therefore, we recommend to add these tests to the initial clinical evaluation of patients with sarcoidosis in order to establish the prognosis and improve the therapeutic approach.

Adult↗
PubMed1996

Periarticular ankle sarcoidosis: a variant of Löfgren's syndrome.

OBJECTIVE: To focus more attention on the syndrome characterized by periarticular ankle inflammation associated with bilateral hilar adenopathy--some authors regard it as a variant of the Löfgren syndrome, while other suggest it is a distinct clinical entity-- we present a series of 33 cases of periarticular ankle sarcoidosis. METHODS: Sarcoidosis was diagnosed in 330 patients at Bellvitge Hospital over a 20 year period. The medical charts of patients who presented with periarticular ankle inflammation (swelling of both ankles with acute inflammatory signs and preserved articular motion) were reviewed. RESULTS: 33 patients (10%) were identified. Periarticular ankle inflammation began during the spring in more than one-half (54.5%). The mean age was 33 years and patients were predominantly women (66.6%). Most cases (78.8%) were stage I on chest radiograph. All patients had thoracic gallium scans showing increased hilar/mediastinal uptake. Increased characteristic parotid, lacrimal, and/or submandibular uptake was found in 11 of 13 patients who had head scans. Four of 6 whose scans included the legs had bilateral ankle uptake. Erythema nodosum was present concomitantly in 36.3% of patients. No granulomas were found in 4 biopsies of periarticular ankle tissue. All 24 patients who were followed had inactive disease one year after diagnosis. CONCLUSION: The association of periarticular ankle inflammation with bilateral hilar adenopathy is an acute form of sarcoidosis that follows a benign course to total remission. It should be regarded as a variant of the Löfgren syndrome.

Adult↗
PubMed1996

Epidemiology of sarcoidosis in Catalonia: 1979-1989.

For the purpose of estimating the incidence rate and clinico-epidemiological characteristics of sarcoidosis in Catalonia (a country comprising the provinces of Barcelona, Tarragona, Lleida and Girona, with a total population of 6 million people), we did a retrospective study of new cases diagnosed in the country's qualified hospitals during 1979 and the three-year period from 1986 to 1988. The annual incidence rate in the province of Barcelona was 1.2/100,000 in 1979 and 1.5/100,000 in 1988. 245 cases were diagnosed in Catalonia in the three-year period covering 1986-88, the annual incidence rate being 1.36/100,000 inhabitants. We describe here the characteristics of this series of 245 cases (which is the first one representative of the whole of Catalonia), and compare them with those of other series published previously. We conclude that the incidence of sarcoidosis in our country is one of the lowest in Europe, with no significant variations observed over ten years. The most relevant clinico-epidemiological characteristics are its clear predominance in women (3:1), and the high frequency of Löfgren's syndrome (48% of cases), whose incidence is significantly higher in springtime.

Adolescent↗
PubMed1995

[The Kveim-Siltzbach test in sarcoidosis].

BACKGROUND: The Kveim-Siltzbach test has been widely used in the diagnosis of sarcoidosis. This reactive is currently available in the authors' hospitals. Previous experience with this test in the same centers was reviewed to know its diagnostic usefulness. The present is the first series described in Spain. METHODS: From 1977 to 1988 the Kveim-Siltzbach test was performed in 79 patients diagnosed or suspected of having sarcoidosis. The study was carried out as cooperation of the validation process of sarcoid spleen suspensions (lots K12, K12 1/2, K32, K41, K42, K42 1/2 and K50 of the Colindale antigen) prepared in the Standards Laboratory for Serological Reagents in the Royal Brompton Hospital in London. RESULTS: The results of test positivity were determined following the criteria established by Siltzbach. The global sensitivity of the Kveim-Siltzbach test was 78% (CI 95% = 67.8-86.9). Positivity ranged from 84% in subacute sarcoidosis to 61% in chronic sarcoidosis (p = 0.08). Likewise, it was higher in patients with radiologic stage I (84%) and II (81%) with respect to stage III (62%) or the cases with exclusive extrathoracic sarcoidosis (stage 0) (57%) (p = 0.11). No association was found between the Kveim-Siltzbach test positivity and the elevation in serum values of the angiotensin-converting enzyme (p = 0.575). CONCLUSIONS: In the present study the Kveim-Siltzbach test showed high sensitivity in the diagnosis of sarcoidosis. This fact, together with the high specificity observed in other studies, makes the test a very useful tool for the non-invasive diagnosis of sarcoidosis.

Acute Disease↗
PubMed1995

Interstitial nephritis in sarcoidosis: simultaneous multiorgan involvement.

Two cases of sarcoidosis with interstitial nephritis (lymphocytic and granulomatous) are described. In contrast to the majority of reported cases, patients simultaneously exhibited renal failure and multiple extrarenal manifestations, including bone marrow granulomas. Pretreatment gallium-67 gamma scan of the kidneys produced negative results. Patients were treated with steroids. Treatment resulted in a normal renal function after 1 year in the patient with lymphocytic interstitial nephritis. Renal function improved in the patient with granulomatous interstitial nephritis, and a second renal biopsy performed 13 months after diagnosis revealed granuloma disappearance and residual interstitial fibrosis. These cases provide evidence that interstitial nephritis in sarcoidosis may appear simultaneously with multiorgan involvement. The absence of gallium-67 renal intake does not guarantee that the disease is not active in the kidneys.

Female↗
PubMed1995

Splenomegaly in sarcoidosis: a report of 16 cases.

The reported frequency of splenomegaly in sarcoidosis has ranged from 1% to 40%. Splenomegaly has been associated with clinical evidence of more extensive extrathoracic sarcoidosis. In a previous study, we reported that splenomegaly was associated with a poor outcome of the disease. The aim of the present study was to describe the characteristics of the sarcoidosis patients with splenomegaly seen at our institution. Of 284 sarcoidosis patients, followed up at our hospital, 16 (5.6%) had splenomegaly on physical examination. These patients showed other extrathoracic manifestations as well, and all but two showed intrathoracic involvement. Eleven patients received corticosteroid therapy. Splenectomy was done in three patients, but the natural history of their sarcoidosis remained unaltered two years after diagnosis. The results of this study disclosed that patients with splenomegaly had evidence of extensive pulmonary and extrathoracic sarcoidosis with a poor prognosis in spite of steroid therapy.

Adolescent↗
PubMed1994

Combined portal and pulmonary hypertension in sarcoidosis.

A case is reported of a 48-year-old man previously diagnosed as having mitral valvular disease, who was admitted for evaluation of chronic cor pulmonale. Seven years before admission, an intraoperative liver biopsy had shown multiple noncaseating granulomas. The further course was characterized by progressive chronic intrahepatic cholestasis and portal hypertension. Right heart catheterization revealed a mean pulmonary artery pressure of 43 mm Hg and a normal wedge pressure (5 mm Hg). A perfusion lung scan was normal. Open lung biopsy demonstrated noncaseating granulomas and extensive pulmonary fibrosis. To our knowledge, only one case of sarcoidosis with combined portal and pulmonary hypertension has previously been described.

Humans↗
PubMed1994

Clinical factors predicting persistence of activity in sarcoidosis: a multivariate analysis of 193 cases.

The prognosis of sarcoidosis is difficult to establish and it depends mainly on the persistence of activity over time and the degree of functional impairment of the involved organs. The aim of this study was to identify factors predicting persistence of disease activity at diagnosis. In a 14-year period (1974-1987), 209 patients were diagnosed with sarcoidosis at Bellvitge Hospital, a 1,000-bed teaching institution in Barcelona, Spain. One hundred ninety-three patients were followed up and included in the study. Clinical and radiological data were collected at diagnosis and a definition of disease activity was established. A Cox proportional-hazards regression model identified the following variables as independently influencing the persistence of activity: absence of erythema nodosum (risk ratio, RR = 2.37; 95% confidence interval, CI: 1.54-3.66), pulmonary infiltrates in chest x-ray (RR = 1.89, 95% CI: 1.28-2.8), splenomegaly (RR = 3.67, 95% CI: 1.46-9.23), age > or = 40 years (RR = 1.01, 95% CI: 1.006-1.03), and absence of lymphadenopathy in chest x-ray (RR = 2.26, 95% CI: 1.08-4.77). We suggest that the identification of factors predicting persistence of sarcoidosis activity at diagnosis may help to establish the prognosis of the disease and therefore improve the therapeutic approach.

Actuarial Analysis↗