The aim of this study was to evaluate health-related quality of life (HR-QOL) in patients with systemic sclerosis (SSc), to compare it with that of patients with rheumatoid arthritis (RA), and to correlate it with other parameters. HR-QOL was evaluated by the Short Form 36 (SF-36), SSc disease activity and severity by preliminary indexes recently proposed, disability by the Health Assessment Questionnaire (HAQ), and depressive symptoms by the Beck Depression Inventory. HR-QOL perception was not statistically different in patients with SSc and RA, except that patients with diffuse cutaneous involvement had worse scores in the general health and mental health dimensions than patients with RA (p=0.03). Compared with RA, patients with SSc tended to perceive less bodily pain (p=0.06) and have less disability (p=0.04) but to report higher depressive symptom scores (p=0.05). SSc patients' HR-QOL was associated with some disease severity scales (general, kidney and, less significantly, heart), but it was poorly correlated with the other evaluated disease activity and severity indexes. A strong correlation with disability and with depressive symptoms was observed. In conclusion, patients with SSc perceived a reduced HR-QOL similar to that of patients with RA. SF-36 may provide useful information in their evaluation.

Iloprost is useful in the short-term treatment of severe Raynaud's phenomenon and ischaemic ulcers in patients with systemic sclerosis (SSc), but its long-term effects are largely unknown. The aim of this study was to report long-term outcome (median follow-up 36 months) in a prospective observational study of a cohort of 30 consecutive patients with SSc who received iloprost therapy with maintenance infusions every 3 weeks after an initial cycle of 5 consecutive days. At the end of the observation, compared to the pretreatment point, we observed complete healing of digital ulcers in 19/21 patients (90%), a decrease of the Raynaud's phenomenon visual analogue score from 10/10 (25th-75th percentile 7-10) to 5/10 (4-6.75) ( P <0.001) and, in patients with diffuse cutaneous involvement, of the modified Rodnan skin thickness score from 25.5 (16.5-31.5) to 16 (13.5-20) ( P = 0.02), minimal improvement of the Health Assessment Questionnaire from 0.87 (0.68-1.37) to 0.75 (0.62-1.25), which was neither statistically nor clinically significant. The forced vital capacity was not significantly changed, but the diffusion capacity corrected for the alveolar volume decreased from 71% (54-76.7) of the expected value to 62% (51.5-71) ( P = 0.02). In one patient with limited SSc a positive effect on pulmonary hypertension was observed. Six patients, after a median of 25 months of treatment and healing of digital ulcers, discontinued the therapy; after a median of 10 months ulcers did not recur in five of these six. Other reasons for discontinuation were: tolerability (1), disease progression (normotensive renal crisis: 1), and death due to intracranial haemorrhage (1). This same patient had previously suffered a central retinal vein thrombosis. In conclusion, long-term therapy with iloprost in patients with SSc has a durable effectiveness on ischaemic ulcers and Raynaud's phenomenon, but it is not possible to conclude that the natural history of the disease was modified.

Fibromyalgia is a chronic widespread pain disorder in which, the neurogenic origin of the pain, featured by allodynia and hyperalgesia, results from an imbalance in the levels of neurotransmitters and consequently of the peripheral pro- and anti-inflammatory mediators. Whole body cryotherapy is a peculiar physical therapy known to relieve pain and inflammatory symptoms characteristics of rheumatic diseases, through the regulation of the cytokine expression. The aim of this study was to qualitatively evaluate the effects of cryotherapy on the clinical output of fibromyalgic patients. A total of 100 fibromyalgic patients (age range 17-70 years) were observed; 50 subjects were addressed to cryotherapy, while the second group (n = 50) did not underwent to the cryotherapic treatment. All subjects kept the prescribed pharmacological therapy during the study (analgesic and antioxidants). The referred health status pre- and post-observation was evaluated with the following scales: Visual Analogue Scale, Short Form-36, Global Health Status and Fatigue Severity Scale. Fibromyalgic patients treated with cryotherapy reported a more pronounced improvement of the quality of life, in comparison with the non-cryo treated fibromyalgic subjects, as indicated by the scores of the qualitative indexes and sub-indexes, that are widely recognized tools to assess the overall health status and the effect of the treatments. We speculate that this improvement is due to the known direct effect of cryotherapy on the balance between pro- and anti-inflammatory mediators having a recognized role in the modulation of pain.

Chilblain lupus erythematosus (CL) of Hutchinson is a subtype of lupus erythematosus (LE) characterized by erythematous lesions induced by cold, damp climates. A number of patients affected by CL eventually develop features of systemic lupus erythematosus (SLE). We report here 9 patients with chilblain cutaneous lesions, 6 of them were affected by SLE and 2 by SCLE. The onset of CL preceded the diagnosis of LE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the remaining 4 cases. Raynaud's phenomenon and photosensitivity were other prominent clinical features in patients with CL. Nailfold capillaroscopy revealed pathological changes in every patient examined. ANA and anti-SSA/Ro antibodies were detected in all nine patients. Anti-SSB/La were detected in 2 cases, anti-Sm in one case, and anti-Sm and anti-RNP in a one case. Antibodies to dsDNA and complement consumption were found in the six patients with SLE. The fine specificity of anti-SSA/Ro was determined by immunoblotting: anti-60kD and anti-52 kD were detected in three sera, anti-60kD alone in 5 sera, while one serum did not blot. In conclusion, the present study suggests that chilblain LE is associated with SSA/Ro autoantibodies, as is SCLE, hypergammaglobulinemic purpura and neonatal lupus erythematosus.

A hypercalcemic condition can be observed in association with hyperthyroidism. The case of a patient suffering from hypercalcemia and hyperthyroidism is reported. A confusional state and EEG alterations, among which diffuse monomorphic delta rhythms were remarkable, are shown. As soon as normalization of calcemia was achieved, a rapid clinical and EEG improvement took place. A hypothetical interpretation is proposed, according to which a prolonged, though inconstant, and mild hypercalcemia in the course of hyperthyroidism could determine an encephalopathy, concealing in some way thyrotoxic symptoms.

BACKGROUND:Accurate clinical restaging is required to select patients who respond to neoadjuvant chemoradiotherapy for locally advanced rectal cancer and who may benefit from an organ preservation strategy. OBJECTIVE:The purpose of this study was to review our experience with the clinical restaging of rectal cancer after neoadjuvant therapy to assess its accuracy in detecting major and pathological complete response to treatment. DESIGN:This was a retrospective cohort study. SETTING:This study was conducted at 2 high-volume Italian centers for Colorectal Surgery.PATIENTS: Data were included from all consecutive patients who underwent neoadjuvant therapy and surgery for locally advanced rectal cancer from January 2012 to July 2020. Criteria to define clinical response were no palpable mass, a superficial ulcer <2 cm (major response), or no mucosal abnormality (complete response) at endoscopy and no metastatic nodes at MRI. MAIN OUTCOME MEASURES:The main outcome measures were sensitivity, specificity, positive predictive values, and negative predictive values of clinical restaging in detecting pathological complete response (ypT0) or major pathological response (ypT0-1) after neoadjuvant therapy.RESULTS: A total of 333 patients were included; 81 (24.3%) had a complete response whereas 115 (34.5%) had a pathological major response. Accuracy for clinical complete response was 80.8% and for major clinical response was 72.9%. Sensitivity was low for both clinical complete response (37.5%) in detecting ypT0 and clinical major response (59.3%) in detecting ypT0-1. Positive predictive value was 68.2% for ypT0 and 60.4% for ypT0-1. LIMITATIONS:The main limitation of the study its retrospective nature.CONCLUSION: Accuracy of actual clinical criteria to define pathological complete response or pathological major response is poor. Failure to achieve good sensitivity and precision is a major limiting factor in the clinical setting. Current clinical assessments need to be revised to account for indications for rectal preservation after neoadjuvant chemoradiotherapy. See Video Abstract at http://links.lww.com/DCR/C63.

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Background Patients suffering from fibromyalgia (FM) had widespread musculoskeletal pain and stiffness, fatigue, sleep disorders, cognitive impairment and other symptoms, which seriously affects their quality of life (QoL), making it difficult to perform normal activities. Treatment of fibromyalgia is multidisciplinary, with an emphasis on active patient participation, medications, cognitive-behavioral therapy and physical modalities. But no single medication has yet been found to sufficiently control all the symptoms of FM, overall pain and fatigue. Whole Body Criotherapy (WBC) administered at –60°C/–140°C has suggested in several study a pain relief in rheumatic diseases associated to increased muscle strength and endurance. Objectives To evaluate the efficacy and the safety of WBC in patients with fibromyalgia. The primary efficacy outcome was defined as the change from baseline (Week 0) to Week 7 in patients’ evaluation of pain, measured by a 100-mm VAS (0 = “No Pain” and 100 = “Maximum Pain”). The secondary efficacy measure was the improvement in Fatigue questionnaire (FSS). Methods 49 randomly fibromyalgic patients (46 F; 3 M; mean age =37,7), meeting both 1990 ACR criteria and Wolfe criteria, using antioxydants agents and analgesics, have been included in this study. They underwent to medical examination and electrocardiogram to determine the suitability and the absence of absolute contraindications to WBC. Each of 49 patient underwent a cycle of 15 therapeutic sessions (5 consecutive days per week, for 3 consecutive weeks). At each session, just before the entrance into the cryochamber and after the therapy, each patient has been monitored for blood pressure, heart rate, oxygen saturation and axillary temperature. Patients stay about 1 minute at a temperature of – 60°C and, immediately after, about 2 minutes at – 140°C and wear a swimsuit, a mask without metals, wool gloves and socks, cover-ears and wooden clogs. At the end of the session patients underwent to specific rehabilitation treatment, at room temperature, for about 30 minutes. At the beginning and four weeks after the end of the cycle of WBC, patients are administered VAS pain and Fatigue questionnaire (FSS). The clinician performs tallying tender points. A second control group of 49 fibromyalgic patients (45 F; 4 M; mean age =39,2) also treated with antioxydants agents and analgesics underwent the same tests. No drug variations were admitted in the two groups during the 7 weeks of observation. Results Whole Body Cryotherapy was significantly superior (P <0.05) to the treatment with only antioxydants agents and analgesic, using the primary criterion (visual analog scale [VAS] assessment of pain). Significant improvement (P <0.05) was also observed for the secondary criteria (Fatigue score). No variations were registered in blood pressure, heart rate, oxygen saturation and axillary temperature. As in international experience we have not reported serious adverse reactions such as permanent injury and/or death. Conclusions Whole Body Cryotherapy...