Search

Background and Objective: To evaluate the long term efficacy of treatment with intravenous iloprost for severe Raynaud's phenomenon (RP) and ischemic leg ulcers in patients with autoimmune systemic diseases., Patients and Method: Prospective observational study over 2 years with iloprost (intravenous infusions, 0.5 to 2 ng/kg/min, initial cycle of 5 consecutive days and maintenance infusions during 24 h monthly, lengthened when it was needed) in patients with severe RP and ischemic leg ulcers whithout response to conventional therapy. Treatment was halted in patients with a good response after one year of treatment, with regular clinical controls., Results: We treated 23 patients. Iloprost reduced significantly the mean number (SD) of monthly episodes of RP (150.38 [102.04] initially and 40.05 [78.06] at the end; p < 0.0005), the mean highest duration of episodes of RP (21.86 [26.96] min initially and 7.14 [9.87] min at the end; p = 0.013), the associated pain (p = 0.005), and the mean number of ischemic digital (4.25 [2.86] initially and 0.63 [2.25] at the end; p = 0.003) and leg ulcers (1.67 [0.52] initially and 0.33 [0.52] at the end; p = 0.01). Articular symptoms and inflammatory markers did not improve. Treatment was stopped in 8 patients (in 5 for a very good evolution and in 3 for other causes), and only 1 of them needed to be treated again. Side effects were seen in all cases but always disappeared after slowing infusion., Conclusions: Iloprost was effective in the long term treatment of severe RP and ischemic leg ulcers in patients with autoimmune systemic diseases.

Background and Objective: Here we report the experience obtained from a combined treatment with intravenous (i.v) prostacyclin and oral sildenafil in patients with severe pulmonary hypertension (PHT) who had a poor response to prior treatment with prostacyclin alone., Patients and Method: Sildenafil was added to the treatment in four patients with PHT (primary in two patients and secondary to collagenosis in the other two) with no adequate response to i.v. prostacyclin treatment. The clinical course, 6minutes walking test and echocardiogram were evaluated., Results: Initial sildenafil dose was 12.5 mg three times daily, which was increased up to 50 mg three times daily in one patient and up to 50 mg four times daily in the other three. The symptoms of right heart failure were controlled in all cases. Before the start of sildenafil administration, two patients had class III dyspnea and two patients had class IV dyspnea. Two patients converted to class I (previously class III and IV), and the other two converted to class II. The distance walked within 6 minutes increased (average increase 55%) and systolic pulmonary artery pressure decreased in all patients (average reduction 27%). Effects of sildenafil were substained. The only side effect seen was mild headache., Conclusions: Our experience supports the value of sildenafil in the treatment of PHT and suggests that combined treatment is useful for rescuing patients who fail to respond to initial treatment with i.v. prostacyclin.

Iliopsoas muscle abscess (IPA) is an uncommon condition, and it is usually associated with immunosuppression. Three out of a cohort of 552 patients diagnosed of systemic lupus erythematosus (SLE) developing an IPA, are reported herein. Patients showed fever and other symptoms related to SLE. They improved only partially under SLE therapy, and showed pain suggestive of IPA. It was confirmed by CT in all cases. S. aureus was isolated in one patient (primary IPA), and M. tuberculosis in the others. Specific antimicrobial therapy and surgical drainage were required. In summary, SLE might be considered as a risk condition for the development of IPA, due to the immunosuppression inherent in the disease and its treatment.

Background: The purpose of this study was to establish the usefulness of single photon emission computed tomography (SPECT) and psychological tests for diagnostic of neuro-Behçet (NB) and to evaluate the clinical significance of neurological symptoms that are difficult to interpret and asymptomatic abnormalities in diagnostic tests., Patients and Method: Forty patients with Behçet's disease (BD) were enrrolled for being studied with magnetic resonance imaging (MRI), SPECT and psychological tests., Results: MRI findings were abnormal in 52,9% of patients with neurological involvement and 23.1% without it (p < 0.1), whereas SPECT findings were abnormal in 82.3% and 61.5%, respectively (no significant difference). The difference between MRI and SPECT findings was significant (p < 0.02 for the complete group; p < 0.05 for patients without neurological symptoms; p < 0.08 for patients with them). The mean follow-up period was 42.6 months, and no patient without neurological involvement or those only with neurological symptoms that are difficult to interprete developed definite neurological involvement. The results of cognitive tests were not significantly different among patients with or without neurological involvement, neither among patients and controls. The scale 2 (depression) of the personality test was more frequent in patients with definite neurological involvement (p < 0.05)., Conclusions: SPECT seems more sensible and less specific than MRI for diagnostic of NB. Although SPECT findings were frequently abnormal in patients with BD without neurological involvement or with neurological symptoms hard to interpret, no patient from this group developed a NB flare after a long follow-up period. A characteristic personality was found for patients with BD.

Werner syndrome or adult progeria is a very rare disease transmitted in a recessive autosomic way. It is characterized by a pathologic and premature aging in all organs and systems, which begins generally between 1st and 3rd decades of life. We discuss a new case with a very visible expression both clinical as well as analytical, highlighting the characteristic chromosomic study.