Sergio Paira

Metabolic syndrome in Argentinean patients with systemic lupus erythematosus

The objective was to determine the prevalence of the metabolic syndrome (MS) in patients with systemic lupus erythematosus (SLE) in Argentina, to assess the factors associated to it, and to compare the results with a control group with non-inflammatory disorders. The study included 147 patients with SLE and 119 controls. MS was defined according to criteria by the American Heart Association/National Heart, Lung, and Blood Institute (AHA/NHLBI) Scientific Statement. Demographic characteristics, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI) were assessed as well as administration, maximum dose and cumulative dose of prednisone and hydroxychloroquine (HCQ). MS prevalence was 28.6% (CI 95%: 21.4–36.6) in patients with SLE and 16% in controls (P = 0.0019). Patients with SLE presented higher arterial hypertension frequency compared with controls (43 vs 25%, P = 0.007). When comparing lupus patients with MS (n = 41) and without MS (n = 106), no significant differences were observed regarding duration of the disease, SLEDAI or cumulative prednisone dose. Cumulative damage was associated independently with MS (OR 1.98; P = 0.021), whereas HCQ use was found to be protective (OR 0.13; P = 0.015). Patients with lupus presented higher MS prevalence than controls with non-inflammatory disorders, and occurrence of arterial hypertension was also higher. MS was associated with cumulative damage; the use of HCQ showed to be protective against presence of MS.

Remitting Seronegative Symmetrical Synovitis with Pitting Oedema: A Study of 12 Cases

Abstract: Twelve patients with remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) were analysed. Eight of them had typical RS3PE without underlying disease, and four presented associated neoplasia. The first patients experienced an excellent response to low doses of prednisone, and they all achieved complete and permanent remission. The mean treatment duration was 18 months and the mean follow-up was 4.4 years. During the follow-up, none of these patients relapsed, had fever or general health deterioration, and hand and foot radiographs did not show erosion. One of them developed a panarteritis nodosa 6 years later. Four RS3PE patients had associated neoplasia. Two were with solid malignancies, and the other two presented haematological malignancies. In one of them RS3PE preceded the diagnosis of malignancy. The diagnosis of RS3PE in the other patients was subsequent to cancer. The first patients presented clinical characteristics suggestive of paraneoplastic RS3PE, and they had a poor response to corticosteroid therapy. Two patients died, and the rest of them had a complete response to surgical resection of the tumour or to chemotherapy. In general, idiopathic RS3PE patients do not show either general health deterioration or fever and they do respond to low doses of steroids (10 mg/day). We observed strong contrasts with the results obtained when treating RS3PE patients with associated neoplasia. In patients with RS3PE the presence of systemic symptoms along with resistance to low doses of corticosteroid therapy should alert the physician to the possible presence of malignancy.

Asymptomatic sensorineural hearing loss in patients with systemic lupus erythematosus.

Objective:Hearing loss can accompany systemic lupus erythematosus (SLE). The purpose of this study was to evaluate the prevalence of asymptomatic sensorineural hearing loss (ASNHL) in patients with SLE. Methods:Thirty-one unselected consecutive female patients with SLE (American College of Rheumatology criteria, 1982) were evaluated (in a prospective and descriptive study) for evidence of hearing abnormalities. Twenty-five healthy age-matched women served as controls. All patients and control groups underwent both a normal tympanoscopy and an audiometric testing as a prerequisite to be included in the study. Results:Patients with SLE had a mean age of 35 years (range, 19–64 years) and the follow-up time (median) was 48 months (range, 4–180 months). One of 31 patients was excluded because of middle ear infectious disease. Of the remaining 30 patients, 21 (70%) had impaired hearing; 20 (66%) had sensorineural loss at high frequencies in a bilateral and symmetric way, and one had conductive alteration. Ten patients had normal audiometric studies. Four women in the control group had alterations of the audiometric tests: 3 patients had conductive alteration and the other one had bilateral ASNHL. No statistically significant correlation was found among the presence of ASNHL, the detection of antiphospholipid antibodies, and the treatment with hydroxychloroquine. Also, no correlation was observed between impaired hearing and SLE activity. Conclusion:If it can be established how often this ASNHL progresses to a clinical problem, it can be important that, as part of initial studies, patients with SLE undergo audiometric tests.

Differential Features Between Primary Ankylosing Spondylitis and Spondylitis Associated with Psoriasis and Inflammatory Bowel Disease

Objective. To describe differential characteristics of axial involvement in ankylosing spondylitis (AS) as compared with that seen in psoriatic arthritis (PsA) and inflammatory bowel disease (IBD) in a cohort of Ibero-American patients. Methods. This study included 2044 consecutive patients with spondyloarthritis (SpA; ESSG criteria). Demographic, clinical, disease activity, functional ability, quality of life, work status, radiologic, and therapeutic data were evaluated and collected by RESPONDIA members from different Ibero-American countries between June and December 2006. Patients selected for analysis met modified New York criteria (mNY) for AS. Results. A total of 1264 patients met the New York criteria for AS: 1072 had primary AS, 147 had psoriatic, and 45 had IBD-associated spondylitis. Median disease duration was comparable among the 3 patient groups. Patients with primary AS were significantly younger (p = 0.01) and presented a higher frequency of males (p = 0.01) than the other 2 groups. Axial manifestations such as inflammatory back pain and sacroiliac pain were significantly more frequent in patients with primary AS (p = 0.05) versus other groups, whereas frequency of dactylitis, enthesitis, and peripheral arthritis was more common in patients with psoriatic spondylitis (p = 0.05). Spinal mobility was significantly more limited in patients with primary AS versus the other 2 groups (p = 0.0001). Radiologic changes according to BASRI total score were equally significant in primary AS. Disease activity (BASDAI), functional ability (BASFI), and quality of life (ASQoL) scores were comparable in the 3 groups. Conclusion. Patients with primary AS had more severe axial involvement than those with spondylitis associated with psoriasis or IBD. Functional capacity, disease activity, and quality of life were comparable among the groups studied.

Peripheral musculoskeletal manifestations in polymyalgia rheumatica.

Objectives:The objectives of this study were to evaluate the frequency and characteristics of the peripheral musculoskeletal manifestations in polymyalgia rheumatica (PMR), evaluate if PMR with peripheral synovitis represents a subset with a more severe disease, and examine for clinical and laboratory characteristics at onset of PMR that might later predict rheumatoid arthritis (RA). Patients and Methods:Patients were diagnosed with PMR according to the 1982 Chuang criteria. Patients were followed up between 1990 and 2002. The following musculoskeletal manifestations at onset and during the follow up were considered: peripheral synovitis, distal extremity swelling with pitting edema, carpal tunnel syndrome, and distal tenosynovitis. Results:Thirty-eight of the 74 patients (51%) showed distal musculoskeletal symptoms: 29 (39%) had peripheral synovitis, 4 (5%) presented pitting edema, 4 (5%) experienced carpal tunnel syndrome, and one (1.3%) had distal tenosynovitis. These manifestations resolved completely after corticosteroid therapy was initiated. Peripheral synovitis was oligoarticular and often transient. The joints most frequently involved were the wrist, metacarpophalangeal, and knee. Erythrocyte sedimentation rate (ESR) was normal in 7 patients. When comparing patients with PMR with and without peripheral synovitis, no statistically significant differences were found in the studied variables. Through the first year of follow up, 7 patients fulfilled the American College of Rheumatology 1987 criteria for RA, 2 patients developed giant cell arteritis, and 3 had associated malignancy. Patients who developed RA had statistically significantly increased presence of persistent synovitis and a smaller decrease in mean ESR after treatment with corticosteroids. Conclusion:Fifty-one percent of the patients with PMR presented distal musculoskeletal manifestations, with peripheral synovitis being the most frequent one. Patients with PMR with peripheral synovitis did not represent a high-risk subgroup with more severe disease. Seven patients who developed criteria for seronegative RA within the first year of follow up had presented statistically significant persistent synovitis compared with those who continued as PMR and also showed a smaller initial decrease in mean ESR after steroid treatment was initiated. The absence of persistent arthritis and the benign course of the arthritis permit the distinction of PMR from other inflammatory arthropathies.

Chromosome 17p12-q11 harbors susceptibility loci for systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the presence of autoantibodies against intracellular components, the formation of immune complexes, and inflammation in various organs, typically the skin and kidney glomeruli. The etiology of the disease is not well understood but is most likely the result of the interaction between genetic and environmental factors. In order to identify susceptibility loci for SLE, we have performed genome scans with microsatellite markers covering the whole genome in families from Argentina, Italy, and Europe. The results reveal a heterogeneous disease with different susceptibility loci in different family sets. We have found significant linkage to chromosome 17p12-q11 in the Argentine set of families. The maximum LOD score was given by marker D17S1294 in combination with D17S1293, when assuming a dominant inheritance model (Z=3.88). We also analyzed a repeat in the promoter region of the NOS2A gene, a strong candidate gene in the region, but no association was found. The locus on chromosome 17 has previously been identified in genetic studies of multiple sclerosis families. Several other interesting regions were found at 1p35, 1q31, 3q26, 5p15, 11q23 and 19q13, confirming previously identified loci for SLE or other autoimmune diseases.

Amerindian ancestry in Argentina is associated with increased risk for systemic lupus erythematosus.

Previous studies have demonstrated that in admixed populations, West African ancestry is associated with an increased prevalence of systemic lupus erythematosus (SLE). In the current study, the effect of Amerindian ancestry in SLE was examined in an admixed population in Argentina. The Argentine population is predominantly European with approximately 20% Amerindian admixture, and a very small (<2%) contribution from West Africa. The results indicate that Amerindian admixture in this population is associated with a substantial increase in SLE susceptibility risk (Odds Ratio=7.94, P=0.00006). This difference was not due to known demographic factors, including site of collection, age and gender. In addition, there were trends towards significance for Amerindian ancestry influencing renal disease, age of onset and anti-SSA antibodies. These studies suggest that populations with Amerindian admixture, like those with West African admixture, should be considered in future studies to identify additional allelic variants that predispose to SLE.

Chronic intestinal pseudo-obstruction in patients with systemic lupus erythematosus: report of four cases

Chronic intestinal pseudo-obstruction (CIPO), a recently recognized manifestation of systemic lupus erythematosus (SLE) with only 23 cases reported in the English literature, may appear as a complication or as the initial presentation of SLE and usually occurs during the setting of an active lupus. The pathogenic mechanism in SLE is unknown. We describe four additional cases with clinical, radiological, and manometric features of CIPO. As SLE-related CIPO usually responds to treatment with high doses of corticosteroids and/or immunosuppressive and prokinetic agents, a high level of awareness of this complication is needed to avoid unnecessary surgical intervention.

Pachydermodactyly: four additional cases.

Pachydermodactyly (PDD) is a rare, benign form of digital fibromatosis characterized by an asymptomatic soft tissue swelling affecting the skin of the lateral aspects of the proximal interphalangeal joints of the fingers, and it sometimes can be misdiagnosed with some rheumatic condition. Recognition on these features should lead to its more frequent diagnosis. The purpose of our study is to report four additional cases of PDD and discuss the differential diagnosis.

Ultrasound nail imaging on patients with psoriasis and psoriatic arthritis compared with rheumatoid arthritis and control subjects.

ObjectiveThe objective of this study was too show findings at finger nails level revealed by high-frequency gray-scale ultrasound (US) and power Doppler in patients with psoriatic arthritis (PsA),and cutaneous psoriasis compared with rheumatoid arthritis and control subjects. MethodsWe studied 35 patients with PsA, 20 with cutaneous psoriasis, and control groups (28 control subjects and 27 patients with rheumatoid arthritis). All nails of both hands were observed (1097 nails, 3 excluded because of trauma). In every patient, we classified the morphologic abnormalities disclosed in ventral and dorsal nail plates. We also measured the distance between ventral plate and the bone margin of the distal phalanx at the right index finger. ResultsAll patients and control subjects presented abnormalities in the US imaging. Those with psoriatic arthritis and cutaneous psoriasis showed a higher number of compromised nails. When classifying those abnormalities using the typology of Wortsman et al, patients with psoriatic arthritis showed loosening of the borders of the ventral plate (type II), whereas patients with cutaneous psoriasis showed focal hyperechoic involvement of the ventral plate without involvement of the dorsal plate (type I). Patients of the control group could not be classified, although 31 of 55 showed thinning of the ventral plate without hyperechoic deposits. Nineteen of 35 patients with psoriatic arthritis, and 10 of 20 patients with cutaneous psoriasis did not show any nail clinical alterations. Nevertheless, the US detected type II alterations in the first group and type I in the second group. Patients with psoriatic arthropathy had power Doppler increases in distal interphalangeal joints and nail beds in a statistically significant form (P = 0.0001).When measuring the distance between the ventral plate and the bone margin of the distal phalanx, there was homogeneity among samples in patients and control subjects. A receiver operating characteristic curve analysis determined that a cut point of 2 mm clearly defined these 2 groups. There was a significant difference (P < 0.0001) between the mean distance ventral plate-osseous margin of the distal phalanx in psoriatic arthritis patients (P = 0.001) and patients with cutaneous psoriasis (P = 0.005) versus rheumatoid arthritis patients (P = 0.548). ConclusionsAs a predominant finding in our study, we observed abnormalities of the ventral plate in patients with PsA (type II) and abnormalities (type I) in patients with cutaneous psoriasis. We found a significant difference between the mean distance ventral plate-osseous margin of the distal phalanx in patients with PsA and patients with cutaneous psoriasis versus control subjects. Ultrasound imaging could be a feasible and sensitive tool to describe, measure, and follow morphologic characteristics and changes of the nail in psoriatic and/ or psoriatic arthritis patients with or without clinical nail lesions.