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.

[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.

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.

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.

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.