Analysis of disease activity and response to treatment in a large Spanish cohort of patients with systemic lupus erythematosus.

OBJECTIVES: The objectives of this paper are to study the impact of disease activity in a large cohort of patients with systemic lupus erythematosus (SLE) and estimate the rate of response to therapies. METHODS: We conducted a nationwide, retrospective, multicenter, cross-sectional cohort study of 3658 SLE patients. Data on demographics, disease characteristics: activity (SELENA-SLEDAI), damage, severity, hospitalizations and therapies were collected. Factors associated with refractory disease were identified by logistic regression. RESULTS: A total of 3658 patients (90% female; median SLE duration (interquartile range): 10.4 years (5.3-17.1)) were included. At the time of their last evaluation, 14.7% of the patients had moderate-severe SLE (SELENA-SLEDAI score ≥6). There were 1954 (53.4%) patients who were hospitalized for activity at least once over the course of the disease. At some stage, 84.6% and 78.8% of the patients received glucocorticoids and antimalarials, respectively, and 51.3% of the patients received at least one immunosuppressant. Owing to either toxicity or ineffectiveness, cyclophosphamide was withdrawn in 21.5% of the cases, mycophenolate mofetil in 24.9%, azathioprine in 40.2% and methotrexate in 46.8%. At some stage, 7.3% of the patients received at least one biologic. A total of 898 (24.5%) patients had refractory SLE at some stage. Renal, neuropsychiatric, vasculitic, hematological and musculoskeletal involvement, a younger age at diagnosis and male gender were associated with refractory disease. CONCLUSIONS: A significant percentage of patients have moderately-to-severely active SLE at some stage. Disease activity has a big impact in terms of need for treatment and cause of hospitalization. The effectiveness of the standard therapies for reducing disease activity is clearly insufficient. Some clinical features are associated with refractory SLE.

Comparison of prevalence of synovitis by ultrasound assessment in subjects exposed or not to self-reported physical overexertion: the Monday's synovitis.

OBJECTIVE: To compare the proportion of synovitis detected by ultrasonographic study (USS) of the hands, in subjects with no rheumatologic known disease according to self-reported level of overexertion performed the day before. METHODS: 407 consecutive volunteers were enrolled in a twelve-month period and underwent an ultrasound assessment of the hand. All studies were performed on Monday or Friday. Subjects were grouped according to their self-reported overexertion carried out the day before. Presence or absence of ultrasonographic findings compatible with synovitis was compared between groups. RESULTS: 95.8% of those tested on Friday had made no overexertion the day before the study, while 30.2% of those assessed on Monday declared to have carried out an overexertion. Presence of carpal synovial hypertrophy, synovial fluid/effusion, and power-Doppler signal was statistically higher in subjects who carried out an overexertion the day before the study than the rest of subjects when the dominant hand was assessed. Globally, presence of any synovitis ultrasonographic finding was statistically higher in subjects who were studied on Monday than Friday (34.9% versus 12.1%) and in subjects who self-reported an overexertion the day before compared to the rest of subjects (47.7 versus 11.5%). CONCLUSIONS: In general, we recommend performing the USS as many days as possible after the most recent overexertion.

Long-term experience of bosentan for treating ulcers and healed ulcers in systemic sclerosis patients.

OBJECTIVES: Our objective was to evaluate the efficacy and tolerability of bosentan in patients with systemic sclerosis (SSc) who develop ulcers and healed ulcers. We also wanted to analyse the effect of bosentan on other skin and general outcome measurements. METHODS: In the present prospective, observational, non-controlled study, we followed all patients with SSc who started treatment with bosentan for ischaemic ulcers and healed ulcers from January 2003 to June 2006 in our centre. We recorded skin and general outcome measurements at baseline and at 6 months. RESULTS: Fifteen patients were included. After a median follow-up of 24.7 months (range: 4-36), there was a significant decrease in the number of ulcers. A trend towards efficacy was seen in the number of healed ulcers and in the severity of ulcers. No significant effect was seen in other skin and general outcome measurements. Toxicity related to bosentan included mild transitory events and one toxic hepatitis. CONCLUSION: Bosentan may be a safe long-term alternative for treating the recurrence of skin ulcers and healed ulcers in SSc patients.

Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature.

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.

Toxic oil syndrome: a syndrome with features overlapping those of various forms of scleroderma.

Thirty-two toxic oil syndrome (TOS) patients were selected because they presented with scleroderma-like changes and were observed during the first 36 months of evolution of the disease. Initially, these patients presented with a noncardiogenic pulmonary edema, eosinophilia, arthralgia/arthritis, peripheral edema, and myositis. Histologic investigations showed a widespread chronic interstitial infiltrate with lymphocytic vasculitis. They subsequently developed peripheral neuropathy, joint contractures, scleroderma-like changes, Raynaud phenomenon, pulmonary hypertension, sicca syndrome, and liver disease. Biopsy studies during this stage showed fibrosis and obliterating arteriopathy. Late features of TOS are musculoskeletal pain, cramps, livedo reticularis, carpal tunnel syndrome, and digital tuft changes. TOS is a new chemically induced scleroderma-like syndrome with features overlapping those of eosinophilic fasciitis, systemic sclerosis, and forms of localized scleroderma.

[Antiphospholipid antibodies in systemic lupus erythematosus: incidence, significance and relation to lupus nephritis]. / Anticuerpos antifosfolípidos en el lupus eritematoso sistémico: incidencia, significación y relación con la nefritis lúpica.

The prevalence of three different types of antiphospholipid antibody in 88 consecutive patients with systemic lupus were 27.2% for lupus anticoagulant (LAC), 31.8% for anticardiolipin antibody (aCL), and 13.6% for falsely positive serologic tests for syphilis (FPSTS). The three tests were correlated, thus confirming the overlapping specificities of this family of antibodies. Although FPSTS was not associated with any particular manifestation of systemic lupus, aCL correlated with thrombosis (p = 0.0001), thrombopenia (p = 0.009), neuropsychiatric features (p = 0.02) and membranous nephropathy (p = 0.001), while LAC correlated with thrombosis (p = 0.001) and hemolytic anemia (p = 0.04). The previously unreported association between membranous nephropathy and aCL might explain some features of the former, particularly the higher incidence of thromboembolic complications and the poorly known relation with renal vein thrombosis.

[Pseudotumor cerebri and systemic lupus erythematosus]. / Pseudotumor cerebri y lupus eritematoso sistémico.

Pseudotumor cerebri (PC) is a syndrome characterized by intracranial hypertension in the absence of any space-occupying lesion, hydrocephalus, cerebral sinus thrombosis and biochemical or cytological abnormalities in the CSF. PC has ben associated with several factors such as systemic conditions or drugs. We report here the case of a patient who presented with headache, vomiting and blurred vision accompanied by bilateral papilledema and had been diagnosed with systemic lupus erythematosus (SLE) seven years before. Treatment was started with high-dose corticosteroids with rapid resolution of the clinical symptoms and papilledema of the patient.

[Anti-ribosomal antibodies in systemic lupus erythematosus]. / Anticuerpos antirribosomales en le lupus eritematoso sistémico.

OBJECTIVES: To compare indirect immunofluorescence (IIF) with immunoblotting (IB) in the detection of antiribosomal antibodies (anti-P Ab) in patients with systemic lupus erythematosus (SLE) and to investigate the possible association between anti-P Ab with serological and clinical findings in SLE, particularly with neurological manifestations. METHODS: Serum specimens from 44 SLE patients and 10 healthy subjects were investigated for anti-P Ab using IB and IIF in rat triple substrate and HEp-2 cells. In SLE patients measurements were made of antinuclear Ab, anti-DNA ds Ab, anti-Sm Ab, anti-U1RNP Ab, anti-Ro Ab, and anti-La Ab. Clinical manifestations of SLE were collected retrospectively when the serological investigation was made. RESULTS: Of the 44 serum specimens tested, 9 showed a ribosomal pattern with triple rat substrate; 8 of them were IB positive (sensitivity 88%; specificity 97%); 12 serum specimens showed a ribosomal pattern with HEp-2 cells by the IIF technique, 9 were positive by IB (sensitivity 100%; specificity 91%). All ten healthy subjects were negative both with IIF and with IB. The nine patients with anti-P Ab in IB (20.45%) had anti-Ro Ab (55% vs. 37%), Anti-Sm Ab (33% vs. 22%, and U1RNP Ab (33% vs. 20%) more frequently than the 35 negative cases. Central nervous system disease (33 vs. 14%), and particularly seizures (33% vs. 5%) and psychosis (22% vs. 8%) were more common in cases with anti-P Ab, but as with serological associations, none of them reached a statistical signification. CONCLUSIONS: IIF with both rat triple substrate and HEp-2 cells is useful for the presumptive diagnosis of anti-P Ab in patients diagnosed with SLE. No significant serological or clinical association was found in patients with anti-P Ab, although neurological disease was more common in these cases.

Rheumatoid nodulosis: report of a case with evidence of intraosseous rheumatoid granuloma.

We describe a patient who had multiple subcutaneous rheumatoid nodules associated with episodes of acute intermittent arthritis and subchondral cystic lesions of the small bones of the hands and feet; this condition is termed "rheumatoid nodulosis." The patient had a cystic lesion in communication with the joint cavity, rheumatoid granulomas, and evidence of a central zone of necrosis opening toward the joint space. His case is compared with 8 previously reported cases, and possible etiologies of the subchondral bone cyst formation in rheumatoid nodulosis are discussed.