Bosentan and sildenafil: successful treatment in a sclerodermic patient with refractory ulcers.

Systemic sclerosis is an inflammatory disease of the connective tissue characterized by vasculopathy and accumulation of collagen and other components of the connective matrix, affecting the skin and internal organs. The appearance of skin ulcers as a result of vascular damage is very common in the history of the disease. Skin ulcers, painful and slow healing due to atrophy and local ischemia, get worse the quality of life of patients. Often, the use of conventional therapies (such as calcium channel blockers and prostanoids) does not cause the complete healing of the lesions. We report the case of a patient in whom therapeutic association between endothelin antagonist (bosentan) and phosphodiesterase-V inhibitor (sildenafil) resulted in complete healing of old ulcers both to upper and lower limbs and allowed the interruption of intravenous therapies.

Thyroid autoimmunity may represent a predisposition for the development of fibromyalgia?

In our previous study, we observed that the presence of autoimmune thyroid disease worsens fibromyalgia (FM) symptoms. The aims of this study are to evaluate whether there is a predisposition for the development of FM in patients with Hashimoto's thyroiditis (HT) with or without subclinical hypothyroidism (SCH) and in patients with SCH alone and what is the weight of antithyroid antibody positivity and SCH on FM comorbidity. Fifty-two patients, 39 affected by HT with or without SCH and 13 by SCH, were matched with 37 patients affected by FM and 25 healthy subjects. Blood samples were collected from all study subjects for the determination of serum TSH, free triiodothyronine, free thyroxine, antithyroperoxidase antibody (TPOAb), antithyroglobulin antibody (TgAb) and non-organ-specific autoantibodies. Clinical assessment of patients and controls included the "Fibromyalgia Impact Questionnaire" (FIQ), while pain severity was evaluated using a visual analogue scale (VAS). Patients and controls were also characterized by the presence of diffuse pain, fatigue, paresthesiae, muscle spasms, non-restful sleep, tension headache and presence of mood disorders. FM comorbidity resulted in twelve HT subjects (31%) and none in SCH patient. In particular, FM comorbidity in HT patients without SCH was 33.3% and in HT patients with SCH was 28.5%. Based on our data, we speculate that maybe there is more than a hypothesis regarding the cause-effect relation between thyroid autoimmunity and the presence of FM, thus suggesting a hypothetical role of thyroid autoimmunity in FM pathogenesis.

[Adherence to cervical cancer screening in an Italian SLE cohort]. / Aderenza allo screening per il cancro della cervice uterina in una coorte italiana di donne affette da LES.

OBJECTIVE: Papanicolau (Pap) smear abnormalities are more frequently observed in systemic lupus erythematosus (SLE) respect to the general population. The primary objective of the present study was to evaluate the adherence to cervical cancer (CC) screening in an Italian cohort of SLE patients and, secondly, to evaluate the disease-related factors possibly influencing the patients' behavior. METHODS: Consecutive 25 to 64 year old SLE patients and aged- matched healthy women were enrolled for the study. All patients were interviewed during ambulatory visits, at admission to the clinic or by a telephone contact; disease related variables were also collected from the clinical charts. RESULTS: 140 SLE patients (mean age 48.3±12 years) and 70 controls matched for demographic and sociocultural characteristics were enrolled. Ninety-three SLE patients (66.4%) declared to perform the Pap test at least every three years (23.6% yearly and 42.8% when asked by the screening programs) while 47 (33.6%) did not perform regular CC screening (16.4% never did the test and 17.1% only occasionally). No significant differences were observed between patients and controls in cancer screening adherence. No significant associations were observed between the screening program behaviors and disease-related variables. CONCLUSIONS: Despite the growing evidence of an increased risk of CC in SLE, and regardless of the broad availability of screening programs and official recommendations, our results show insufficient CC surveillance among SLE patients and emphasize to rheumatologists and/or general practitioners the importance to discuss with patients this aspect during routine evaluations in order to encourage compliance to the recommended preventive measures.

Spasmophilia comorbidity in fibromyalgia syndrome.

OBJECTIVES: To evaluate the role of spasmophilia (SP) in fibromyalgia syndrome (FM). METHODS: Three hundred and fourteen patients (280 F, 34 M) with a diagnosis of FM or FM and spasmophilia (FM+SP) were recruited. Clinical assessment of patients and controls included the Questionnaires FIQ, HAQ and the tender point (TP) count. Life-time or ongoing psychiatric aspects were evaluated by trained psychiatrists by means of the classic scales: Structured Clinical Interview (SCID) for DSM-IV. The following analysis were evaluated: cytokine (IL1, IL2, IL6, IL8, IL10), TNF-α, cortisol, GH, ACTH, IGF1, 5HT, intracellular Mg, plasma calcium p(Ca), PTH, (25(OH)D) and thyroid functionality. Some typical symptoms were investigated. RESULTS: Eighty-one patients resulted positive for spamophilia (FM+SP), while 233 resulted negative for spasmophilia (FM). The mean TP number resulted higher in the FM group (15.33±3.88) with respect to FM+SP (12.88±6.17, p=0.016), while FIQ and HAQ did not differ between the two studied groups. FM patients exhibited a higher frequency of psychiatric disorders with respect to FM+SP patients (72% FM vs. 49% FM+SP, p<0.01). In particular the frequency of depression was 65.5% FM vs. 35% FM+SP (p<0.01). CONCLUSIONS: The presence of spasmophilia seems to influence psychiatric comorbidity which was less prevalent in FM+SP patients. FM is indeed characterised by an abnormal sensory processing of pain that seems to result from a combination of interactions between neurotransmitters, stress, hormones and the nervous system; spasmophilia would seem to be more linked to a dysfunction at the neuromuscular level.

[Ultrasound lung comets: new echographic sign of lung interstitial fibrosis in systemic sclerosis]. / Le comete ultrasoniche polmonari (ULC): un nuovo segno ecografico di fibrosi polmonare nella sclerodermia.

OBJECTIVE: Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are common complications of systemic sclerosis (SSc). Echocardiography evaluates PAH, and chest sonography detects even mild ILC as ultrasound lung comets (ULC), i.e. multiple comet-tails fanning out from the lung surface and originating from subpleural interlobular septa thickened by fibrosis. AIM: to assess ILaD and PAH by integrated cardiac and chest ultrasound in SSc. METHODS: We enrolled 30 consecutive SSc patients (age= 54+/-13 years, 23 females) in the Rheumatology Clinic of Pisa University. In all, we assessed systolic pulmonary arterial pressure (SPAP), from maximal velocity of tricuspid regurgitation flow, and ULC score with chest sonography (summing the number of ULC from each scanning space of anterior and posterior right and left chest, from second to fifth intercostal space). All patients underwent plasma assay for anti-topoisomerase antibodies (anti-Scl70), and antiicentromere associated with development of pulmonary involvement. Twenty-eight patients also underwent high resolution computed tomography, HRCT (from 0= no fibrosis to 3= honey combing). RESULTS: ULC number - but not SPAP - was correlated to HRCT fibrosis and presence Scl-70 antibodies. ULC number was similar in localized or diffuse forms (16+/-20 vs 21+/-19, p=ns) and was unrelated to SPAP (r=0.216, p=ns). CONCLUSIONS: Chest sonography assessment and ULC allow a complete, simple, radiation-free characterization of interstitial lung involvement in SSc - all in one setting and with the same instrument, same transducer and the same sonographer. In particular, ULC number is associated with HRCT evidence of lung fibrosis and presence of Scl-70 antibodies.