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Josep Font

Publications and source records attributed to Josep Font.

At least 55 records · Page 3Linked to original sources
PubMed2005

Circulating monoclonal immunoglobulins in Sjögren syndrome: prevalence and clinical significance in 237 patients.

We conducted the current study to analyze the prevalence and clinical significance of circulating monoclonal immunoglobulins in patients with Sjögren syndrome (SS), focusing on the association with extraglandular features, immunologic markers, hematologic neoplasia, and hepatitis C virus (HCV) infection. We performed serum immunoelectrophoresis in 200 patients with primary SS and 37 patients with HCV-related SS. All patients fulfilled 4 or more of the 1993 European classification criteria for SS.Of the 200 patients with primary SS, 35 (18%) presented circulating monoclonal immunoglobulins. The monoclonal bands identified were 20 IgG (13 kappa, 7 lambda), 10 IgM (5 kappa, 5 lambda), 2 IgAkappa, and 3 free circulating light chains. Of the 37 SS-HCV patients, 16 (43%) had circulating monoclonal immunoglobulins. The monoclonal bands identified were 10 IgMkappa, 5 IgGlambda, and 1 free light lambda chain. Compared with primary SS patients, SS-HCV patients presented a higher frequency of monoclonal immunoglobulins (43% vs 18%, p = 0.001), with monoclonal IgMkappa being the most frequent monoclonal band. Six (12%) of the 51 SS patients with circulating monoclonal immunoglobulins presented hematologic neoplasia, compared with 3 (1.6%) of those without monoclonal immunoglobulins (p = 0.004; odds ratio = 8.13; 95% confidence intervals, 1.64-51.54). In 2 of the 6 patients with monoclonal immunoglobulins and lymphoproliferative disorders, a change of the monoclonal component was detected in previous immunoelectrophoresis determinations before the development of hematologic neoplasia. Circulating monoclonal immunoglobulins were detected in nearly 20% of patients with primary SS, with monoclonal IgG being the most frequent type of immunoglobulin detected. In SS-HCV patients, the prevalence of monoclonal immunoglobulins was higher (43%), with monoclonal IgM being the most frequent type found. SS-HCV patients presented a more restrictive monoclonal expression (limited to either monoclonal IgMkappa or monoclonal IgGlambda) than primary SS patients, who showed all types of heavy and light chains.

Adult↗
PubMed2005

Sarcoidosis in patients with chronic hepatitis C virus infection: analysis of 68 cases.

We describe the clinical characteristics, the patterns of association, and the role of antiviral therapies in patients with sarcoidosis associated with chronic hepatitis C virus (HCV) infection. Sixty-eight patients were included in the current study, 56 cases identified in the literature search plus 12 unpublished cases from our department. In 50 HCV patients, sarcoidosis appeared after starting antiviral therapy. Antiviral therapy associated with triggered sarcoidosis consisted of alpha-interferon monotherapy in 20 cases and combined therapy with alpha-interferon and ribavirin in 30. Sarcoidosis appeared during the first 6 months after starting therapy in 66% of patients. The clinical picture of sarcoidosis included predominantly pulmonary disease in 38 (76%) patients and cutaneous sarcoidosis in 30 (60%). Antiviral therapy was discontinued in 60% of patients and continued or adjusted in 14%, while sarcoidosis appeared after completed therapy in the remaining cases. Specific therapy for sarcoidosis was started in only 21 patients, mainly with oral corticosteroids. The outcome of patients was detailed in 46 cases: remission or improvement was observed in 38/46 (83%) patients, stabilization of sarcoidosis in 5/46 (11%), and reactivation of sarcoidosis after an initial improvement in 3/46 (6%). Finally, 18 treatment-naive HCV patients presented sarcoidosis, with 14/18 (87%) patients presenting with pulmonary involvement and 8/18 (44%) with cutaneous involvement. In summary, sarcoidosis may be observed in HCV patients in 2 different situations: triggered by antiviral therapy (in 75% of cases) and unrelated to treatment. Sarcoidosis during antiviral therapy may present mainly as cutaneous or pulmonary disease, with a benign, uncomplicated evolution in more than 85% of cases. However, more complicated cases are observed, especially in HCV patients with preexisting sarcoidosis and/or with previous antiviral treatment. Clinicians should be aware of the possibility that sarcoidosis may initially manifest or be reactivated during or shortly after treatment with antiviral therapy in patients with chronic HCV infection.

Adult↗
PubMed2005

Class III-IV proliferative lupus nephritis and pregnancy: a study of 42 cases.

PROBLEM: A growing number of women with lupus nephritis wish pregnancy. Our aim was to analyze maternal and fetal outcome in pregnancies with the most severe forms (proliferative or class III-IV) of lupus nephritis. METHOD OF STUDY: Forty-two pregnancies in 35 women with class III or IV lupus nephritis confirmed by renal biopsy (group 1); 12 pregnancies in 10 patients having histologically proven lupus nephritis class II or V (group 2); and 54 pregnant women randomly selected among our cohort of pregnant lupus patients without nephropathy who were matched for age, parity and duration of lupus to patients with class III or IV lupus nephritis (group 3) were studied. RESULTS: Pregnancy outcome and mean gestational age of neonates were similar in the three groups studied with hypertension and preeclampsia, being significantly more prevalent in patients in group 1 (37.1%) than among patients in groups 2 (11.1%) and 3 (11.6%) (P < 0.05). Mean birthweight (+/-S.D.) was significantly lower in group 1 (2214 +/- 802 g) than in groups 2 (2783 +/- 721 g) and 3 (2870 +/- 835 g) (P < 0.05). CONCLUSION: Lupus nephritis class III-IV is a risk factor for hypertensive disease during pregnancy, but it does not contraindicate gestation.

Adult↗
PubMed2005

[Sarcoidosis as seronegative spondyloarthropathy].

Two cases were presented with initial symptoms of inflammatory low back pain and alternate buttock pain. They developed a progressive dyspnea with bilateral mediastinal and hiliar lymphadenopathy and pulmonary interstitial disease as visualized with chest CT scan. Sarcoidosis diagnosis was confirmed by biopsy in both cases--in one case by skin biopsy and in the other by open lung biopsy. These clinical forms of spondyloarthropathy and sarcoidosis were unusual and were compared with similar cases present in the literature.

Adult↗
PubMed2004

Highly efficient and diastereoselective synthesis of (+)-lineatin.

[reaction: see text] A linear sequence was used to synthesize (+)-lineatin in 14 steps and 14% overall yield from a homochiral 2(5H)-furanone. Key steps of this synthetic approach feature the diastereoselective construction of a cyclobutene through a photochemical [2 + 2] cycloaddition and a regiocontrolled oxymercuration reaction.

Cyclization↗
PubMed2004

Clinical features related to antiphospholipid syndrome in patients with chronic viral infections (hepatitis C virus/HIV infection): description of 82 cases.

We analyzed the spectrum of clinical features related to antiphospholipid syndrome (APS) in patients with chronic viral infections, such as hepatitis C virus (HCV) infection and human immunodeficiency virus (HIV) infection. We selected patients from the HISPAMEC registry who repeatedly tested positive for antiphospholipid antibodies (aPL) and who had features of APS, and we searched the MEDLINE database for additional cases. A total of 82 patients were included (45 had chronic HCV infection, 32 had HIV infection, and 5 had HCV-HIV coinfection). The main features of APS were avascular bone necrosis (20 patients), peripheral thrombosis (17), thrombocytopenia (15), neurologic features (13), cardiac manifestations (12), pulmonary embolism (9), gastrointestinal manifestations (8), and cutaneous manifestations (8). The main APS-related features in HCV-infected patients were intraabdominal thrombosis and myocardial infarction, whereas, in HIV-infected patients, the main features were avascular bone and cutaneous necrosis. These viruses might act in some patients as chronic triggering agents that induce a heterogeneous, atypical presentation of APS.

Adult↗
PubMed2004

Early response to immunosuppressive therapy predicts good renal outcome in lupus nephritis: lessons from long-term followup of patients in the Euro-Lupus Nephritis Trial.

OBJECTIVE: In the Euro-Lupus Nephritis Trial (ELNT), 90 patients with lupus nephritis were randomly assigned to a high-dose intravenous cyclophosphamide (IV CYC) regimen (6 monthly pulses and 2 quarterly pulses with escalating doses) or a low-dose IV CYC regimen (6 pulses of 500 mg given at intervals of 2 weeks), each of which was followed by azathioprine (AZA). After a median followup of 41 months, a difference in efficacy between the 2 regimens was not observed. The present analysis was undertaken to extend the followup and to identify prognostic factors. METHODS: Renal function was prospectively assessed quarterly in all 90 patients except 5 who were lost to followup. Survival curves were derived using the Kaplan-Meier method. RESULTS: After a median followup of 73 months, there was no significant difference in the cumulative probability of end-stage renal disease or doubling of the serum creatinine level in patients who received the low-dose IV CYC regimen versus those who received the high-dose regimen. At long-term followup, 18 patients (8 receiving low-dose and 10 receiving high-dose treatment) had developed permanent renal impairment and were classified as having poor long-term renal outcome. We demonstrated by multivariate analysis that early response to therapy at 6 months (defined as a decrease in serum creatinine level and proteinuria <1 g/24 hours) was the best predictor of good long-term renal outcome. CONCLUSION: Long-term followup of patients from the ELNT confirms that, in lupus nephritis, a remission-inducing regimen of low-dose IV CYC followed by AZA achieves clinical results comparable with those obtained with a high-dose regimen. Early response to therapy is predictive of good long-term renal outcome.

Adolescent↗
PubMed2004

Systemic autoimmune diseases in elderly patients: atypical presentation and association with neoplasia.

The possible consequences of the progressive 'ageing' of the immune system are the increase in autoimmune phenomenon, incidence of neoplasia and predisposition to infections. This review analyses the clinical expression of the systemic autoimmune diseases in older patients, focusing on three specific characteristics: the frequent atypical presentation of autoimmune diseases in the elderly, the higher morbidity and mortality of these patients and the frequent association with neoplasic processes. The study of autoimmune manifestations in elderly populations should be considered a priority for future medical research because of increasing life expectation, especially in developed countries.

Aged↗
PubMed2004

Clusters of clinical and immunologic features in systemic lupus erythematosus: analysis of 600 patients from a single center.

OBJECTIVE: To analyze the prevalence and characteristics of the main clinical, hematologic, and immunologic manifestations of systemic lupus erythematosus (SLE) in a cohort of 600 consecutive patients from a single center, and to determine the specific characteristics of organ involvement in a homogeneous SLE population. METHODS: Patients were consecutively seen in our department either as inpatients or outpatients between 1980 and 2001. All had documented medical histories and underwent a medical interview as well as a routine general physical examination. Clinical and serologic characteristics of all patients were consecutively collected in a protocol form. RESULTS: The final cohort (survival cohort) consisted of 533 (89%) women and 67 (11%) men (female to male ratio, 8:1), with an average of 29 new patients per year. Mean age at onset of symptoms attributable to the disease was 31 years (range, 5 to 84 years) and mean age at the time of diagnosis of SLE was 33 years (range, 6 to 85 years). The most frequent SLE involvement was articular involvement, found in 498 patients (83%), followed by hematologic involvement in 451 patients (75%), specific SLE cutaneous involvement in 354 patients (59%), constitutional features in 252 patients (42%), and nephropathy in 203 patients (34%). Patients enrolled in the protocol before 1991 had a higher frequency of central nervous system (CNS) involvement (27% vs 10%, P <.001), thrombotic events (17% vs 9%, P =.003), and abnormal hematologic parameters (85% vs 66%, P <.01), but a lower frequency of articular involvement (79% vs 86%, P =.038) than those enrolled after 1991. The following were observed associations: specific SLE cutaneous involvement was associated with anti-Sm antibodies; renal involvement with hemolytic anemia and anti-double-stranded DNA antibodies; CNS involvement with thrombocytopenia and immunoglobulin G-anticardiolipin; thrombotic events with low total hemolytic complement, immunoglobulin G-anticardiolipin, and lupus anticoagulant; and myositis with anemia and anti-ribonucleoprotein antibodies. CONCLUSION: This large study, performed in a single center, has shown cluster associations between certain clinical, hematologic, and immunologic features of SLE, reflecting specific patterns of disease expression. The accurate evaluation of clinical features and laboratory markers at disease diagnosis and during evolution may improve the clinical treatment of SLE patients.

Adolescent↗
PubMed2004

Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients.

To analyze the different clinical and histologic types of cutaneous vasculitis in patients with primary Sjögren syndrome (SS), we investigated the clinical and immunologic characteristics of 558 consecutive patients with primary SS from our units and selected those with clinical evidence of cutaneous lesions, excluding drug reactions and xeroderma. All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. A total of 89 (16%) patients presented with cutaneous involvement (88 female patients and 1 male; mean age, 51.8 yr). The main cutaneous involvement was cutaneous vasculitis, present in 52 (58%) patients. There were 51 (98%) female patients and 1 (2%) male, with a mean age at diagnosis of cutaneous vasculitis of 51 years (range, 20-80 yr). Fourteen presented with cryoglobulinemic vasculitis, 11 with urticarial vasculitis, and the remaining 26, with cutaneous purpura not associated with cryoglobulins. A skin biopsy specimen was obtained in 38 patients (73%). Involvement of small-sized vessels was observed in 36 (95%) patients (leukocytoclastic vasculitis), while the remaining 2 (5%) presented with medium-sized vessel vasculitis (necrotizing vasculitis). Patients with cutaneous vasculitis had a higher prevalence of articular involvement (50% vs 29%, p = 0.044), peripheral neuropathy (31% vs 4%, p < 0.001), Raynaud phenomenon (40% vs 15%, p = 0.008), renal involvement (10% vs 0%, p = 0.028), antinuclear antibodies (88% vs 60%, p = 0.002), rheumatoid factor (78% vs 48%, p = 0.004), anti-Ro/SS-A antibodies (70% vs 43%, p = 0.011), and hospitalization (25% vs 4%, p = 0.005) compared with SS patients without vasculitis. Six (12%) patients died, all of whom had multisystemic cryoglobulinemia.In conclusion, cutaneous involvement was detected in 16% of patients with primary SS, with cutaneous vasculitis being the most frequent process. The main characteristics of SS-associated cutaneous vasculitis were the overwhelming predominance of small versus medium vessel vasculitis and leukocytoclastic versus mononuclear vasculitis, with a higher prevalence of extraglandular and immunologic SS features. Small vessel vasculitis manifested as palpable purpura, urticarial lesions, or erythematosus maculopapules, with systemic involvement in 44% of patients in association with cryoglobulins in 30%. Life-threatening vasculitis was closely related to cryoglobulinemia.

Adult↗
PubMed2004

Sarcoidosis or Sjögren syndrome? Clues to defining mimicry or coexistence in 59 cases.

We present 5 new cases of coexisting sarcoidosis and Sjögren syndrome (SS) and review the literature for additional cases in order to analyze the clinical, immunologic, and histologic characteristics that may help physicians differentiate the mimicry of SS by sarcoidosis from a true coexistence of both autoimmune diseases. We considered the coexistence of sarcoidosis with SS to be when patients presented specific histologic patterns of both diseases, simultaneously or at different times.Fifty-nine patients were included in the analysis (54 identified in the literature search plus our 5 unpublished cases): 49 (83%) patients were female and 10 (17%) were male, with a mean age at diagnosis of 50 years. According to the histopathologic examination of the exocrine glands performed in 53 cases, we defined coexistence of sarcoidosis and SS in 28 cases, while in the remaining 25 patients, sarcoidosis mimicked SS. Clues to identifying when sarcoidosis coexists with SS were a higher prevalence of systemic manifestations (arthritis and uveitis) and positive immunologic parameters (antinuclear antibodies, rheumatoid factor, and anti-Ro/SS-A), as well as the existence of a focal sialadenitis (Chisholm-Mason score grades III-IV, with a CD4+ lymphocytic infiltration) in the salivary gland biopsy. In patients first diagnosed with primary SS, the appearance of some clinical features such as hilar adenopathies, uveitis, or hypercalcemia leads to the diagnosis of coexisting sarcoidosis. A careful application of the new American-European consensus criteria had a sensitivity of 93% and a specificity of 92% in identifying when SS coexists with sarcoidosis.In conclusion, the association of sarcoidosis with SS leads to a true coexistence of both diseases in more than half the patients described in the literature, while in the remaining patients, sarcoidosis mimics SS. In light of these results, sarcoidosis should not be considered as an exclusion criterion for the diagnosis of SS, and in patients with a suspected overlap of the two diseases, application of the new American-European consensus criteria for diagnosis of SS should be mandatory.

Adult↗
PubMed2004

Differences in open field behavior between heterozygous and homozygous negative gilts for the RYR(1) gene.

A test widely used to assess fear and novelty responses in domestic species is the open field. The aim of this study was to investigate the effect of RYR(1) genotype on open field behavior in growing pigs. The study subjected 15 heterozygous (Nn) and 15 RYR(1)-free (NN) gilts of 19 weeks of age to 3 replicates of an open field test 2 days apart from each other. The study measured the number of grid lines crossed and defecation score in the test arena. There was a significant individual correlation among the 3 replicates of the test, both for number of grid lines crossed and defecation score (p <.05). RYR(1) genotype had a significant effect on number of grid lines crossed, with NN gilts showing more overall activity than Nn gilts (p <.05). The study observed no significant differences in defecation score between genotypes. This result suggests that the RYR(1) genotype may have an effect on the appraisal of novelty. Thus, it would be interesting to take this factor into account when using this methodology to assess fear responses in pigs and in interpreting the results with respect to welfare.

Animal Husbandry↗
PubMed2004

Triple association between hepatitis C virus infection, systemic autoimmune diseases, and B cell lymphoma.

OBJECTIVE: To analyze the clinical characteristics of patients from a Department of Autoimmune Diseases presenting chronic hepatitis C virus (HCV) infection, systemic autoimmune disease, and B cell lymphoma. METHODS: We analyzed the records of 100 consecutive patients with systemic autoimmune diseases and associated HCV infection seen in our department between 1994 and 2000. We retrospectively investigated the development of B cell malignancies after the diagnosis of HCV related autoimmune disease. RESULTS: Six patients with HCV related systemic autoimmune disease presented B cell non-Hodgkin's lymphoma (NHL). These patients fulfilled the diagnostic criteria for Sjögren's syndrome (n = 4) and polyarteritis nodosa (PAN; n = 2). Four patients were female and 2 male, with a mean age at lymphoma diagnosis of 62 years (range 45-78). The main immunologic markers were hypocomplementemia in all patients and cryoglobulinemia in 5 (83%). Primary extranodal localization of lymphoma was observed in 3 (50%) patients: prostate (n = 1), liver and ovary (n = 1), and ocular annexa (n = 1). Clinically, NHL was classified as indolent lymphoma in 3 patients and aggressive lymphoma in 3. NHL histologic types were diffuse large cell lymphoma (n = 4), extranodal marginal zone B cell lymphoma (n = 1), MALT lymphoma (n = 1), and lymphoplasmacytic lymphoma (n = 1). CONCLUSION: We describe 6 patients with a triple association of HCV infection, systemic autoimmune disease, and NHL. Characteristics of these patients included a high prevalence of cryoglobulinemia (that clearly contributes to fulfillment of diagnostic criteria for PAN) and an elevated frequency of primary extranodal involvement. We recommend careful evaluation of patients with B cell NHL to detect silent autoimmune or chronic viral diseases. This triple association reinforces the suspected links between autoimmunity, infection, and cancer.

Adult↗
PubMed2003

C2-Symmetric enantiopure ethanotethered bis(alpha,beta-butenolides) as templates for asymmetric synthesis. Application to the synthesis of (+)-grandisol.

Starting from D-mannitol, we have prepared several C(2)-symmetric ethanotethered bis(alpha,beta-butenolides) and studied their [2+2] photocycloaddition reaction with ethylene. The protective groups of the central diol unit have a noticeable influence on the facial selectivity of the cycloaddition, the bis(trimethylsilyloxy) derivatives showing the highest diastereoselectivity. A theoretical conformational analysis of the substrates in the ground state is in good agreement with the diastereofacial selectivity experimentally observed. The bis(photocycloadducts) have been converted into the enantiopure cyclobutanes formally derived from the photoreaction of ethylene with gamma-hydroxymethyl-alpha,beta-butenolide, in which only a moderate facial selectivity had been previously found. As an application of these studies, we have developed a highly efficient and stereoselective synthesis of (+)-grandisol.

4-Butyrolactone↗