Buerger\'s disease is characterized by peripheral ischemia due to occlusion of small- and medium-sized arteries in the extremities. This report describes a case of Buerger\'s disease in a 51-year-old male who presented with findings resembling systemic sclerosis. The patient exhibited Raynaud\'s phenomenon in year X-3, which developed to skin hardening, nail avulsion, and ulceration of the right fingers in year X. Diagnostic testing showed positive microvasculopathy on nailfold videocapillaroscopy (NVC) and positive fibrosis on skin biopsy. Although the patient fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for systemic sclerosis, several findings in this case were atypical for systemic sclerosis, including left-right asymmetry in finger involvement, nail loss, and negative autoantibody tests. Contrast-enhanced computed tomography showed poor perfusion of the right ulnar artery, and a heavy smoking history was established in the patient case. Therefore, based on Shionoya\'s criteria, he was diagnosed with a case of Buerger\'s disease confined to the upper extremity. Smoking cessation and vasodilator therapy resulted in the prompt resolution of ischemic symptoms, skin hardening, and ulcerations. Furthermore, NVC abnormalities improved, and ulnar artery occlusion showed reperfusion on repeat testing. The present case suggests that hypoxemia-driven microvasculopathy may contribute to vascular occlusion and skin fibrosis observed in this atypical presentation.

Raynaud\'s phenomenon (RP) is a condition characterized by episodic, excessive vasoconstriction in the fingers and toes, triggered by cold or stress. This leads to a distinctive sequence of color changes in the digits. Pallor indicates reduced blood flow due to oxygen deprivation, while erythema appears as reperfusion. RP can be primary, with no identifiable underlying cause, or secondary, associated with other conditions. These conditions include autoimmune diseases, most commonly systemic sclerosis, vascular diseases; and neurological conditions. While the exact cause of RP remains unclear, genetic and hormonal (estrogen) factors are likely contributors. The pathogenesis of RP involves a complex interaction between the vascular wall, nerves, hormones, and humoral factors, disrupting the balance between vasoconstriction and vasodilation. In primary RP, the vascular abnormalities are primarily functional. However, in secondary RP, both functional and structural components occur in blood vessels. This explains why digital tissue damage frequently occurs in secondary RP but not primary RP. Diagnosis of RP is primarily clinical. Recent advancements in imaging techniques have aided in diagnosis and monitoring, but nail fold capillaroscopy remains the gold standard for distinguishing between primary and secondary RP. If there are signs of acute ischemic injury, vascular imaging, particularly preoperatively, is crucial to rule out other vaso-occlusive conditions. Management of RP focuses on alleviating symptoms and preventing tissue damage. Vasodilator medications are the first-line treatment when general measures like warmth and stress management are not sufficient. Dihydropyridine calcium channel blockers (CCBs), such as nifedipine, are commonly used for vasodilation. Phosphodiesterase-5 inhibitors and prostaglandin analogs are alternative options for patients who do not respond to CCBs or have ischemic tissue damage. Bosentan, an endothelin-1 receptor antagonist, has shown effectiveness in treating and preventing digital ulcers, especially in patients with multiple ulcers. For severe cases, botulinum toxin injections or sympathectomy surgery can be used to control RP symptoms. However, botulinum toxin injections require repeated administration, and sympathectomy\'s long-term effectiveness is uncertain. Fat grafting is a promising surgical therapy for promoting healing and preventing tissue injury.

BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc.
METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed.
RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud\'s phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found.
CONCLUSIONS: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.

UNASSIGNED: To investigate the hypotheses that in patients with SSc, the temperature gradient between the dorsum of the foot and toes (distal-dorsal difference [DDD]) is \'more negative\' (toes cooler) than in healthy controls, is greatest along the first (great) toe and that the severities of thermographic abnormalities in the feet and hands are correlated.
UNASSIGNED: Thermographic images of the dorsum of each hand and foot were captured using a thermal camera attached to an iPhone in 40 patients with SSc and 20 healthy controls. DDDs along the fingers (index, middle, ring and little) and toes (great toe and \'others\') were measured.
UNASSIGNED: There was a non-significant trend for the great toes to be colder in patients with SSc than in controls. The mean great toe DDD was more negative in patients (right: -2.89°C, left: -2.91°C, mean: -2.90°C) than in controls (right: -2.36°C, left: -2.42°C, mean: -2.39°C) (P = 0.37 for mean values). Patients\' great toes were colder than \'other\' (lesser) toes (right: -2.58°C, left: -2.63°C), although not significantly. In patients with SSc, finger and great toe temperature gradients were correlated (r = 0.406, ρ = 0.01).
UNASSIGNED: Our findings suggest that the great toe is the coldest in patients with SSc and that patients with the coldest fingers tend to have the coldest toes. Severe RP symptoms in the hands should prompt podiatry assessment and foot care education. Mobile phone thermography is a convenient tool for assessing the digital vasculature but first requires validation in larger studies with a longitudinal component.

A 50-year-old female patient presenting with joint pains, Raynaud\'s phenomenon, epistaxis, and telangiectasias was posed with a diagnostic conundrum, i.e., whether to accept the diagnosis of mixed connective tissue disease (MCTD), for which she fulfilled all the criteria, or test for another probable disease, namely hereditary hemorrhagic telangiectasia (HHT), even though only some clinical features were present and all diagnostic criteria were not satisfied. Taking the patient\'s onset of epistaxis as an important clue, the patient was counseled for genetic testing for HHT, which was positive. Treatment for both MCTD and HHT is underway, and appropriate surveillance is planned for the patient.

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OBJECTIVE: Our objective was to test the hypothesis, in a double-blind, placebo-controlled study that vipoglanstat, an inhibitor of microsomal prostaglandin E synthase-1 (mPGES-1) which decreases prostaglandin E2 (PGE2) and increases prostacyclin biosynthesis, improves RP.
METHODS: Patients with systemic sclerosis (SSc) and ≥7 RP attacks during the last screening week prior to a baseline visit were randomised to four weeks treatment with vipoglanstat 120 mg or placebo. A daily electronic diary captured RP attacks (duration and pain) and Raynaud\'s Condition Score, with change in RP attacks/week as primary end point. Cold challenge assessments were performed at baseline and end of treatment. Exploratory endpoints included patients\' and physicians\' global impression of change, Assessment of Scleroderma-associated Raynaud\'s Phenomenon questionnaire, mPGES-1 activity, and urinary excretion of arachidonic acid metabolites.
RESULTS: Sixty-nine subjects received vipoglanstat (n = 33) or placebo (n = 36). Mean weekly number of RP attacks (baseline; vipoglanstat 14.4[SD 6.7], placebo 18.2[12.6]) decreased by 3.4[95% CI -5.8;-1.0] and 4.2[-6.5;-2.0] attacks per week (p= 0.628) respectively. All patient reported outcomes improved, with no difference between the groups. Mean change in recovery of peripheral blood flow after cold challenge did not differ between the study groups. Vipoglanstat fully inhibited mPGES-1, resulting in 57% reduction of PGE2 and 50% increase of prostacyclin metabolites in urine. Vipoglanstat was safe and well tolerated.
CONCLUSIONS: Although vipoglanstat was safe, and well tolerated in a dose achieving full inhibition of mPGES-1, it was ineffective in SSc-related RP. Further development and evaluation of vipoglanstat will therefore be in other diseases where mPGES-1 plays a pathogenetic role.

BACKGROUND: Despite emotional stress being recognised as a key trigger for Raynaud\'s phenomenon episodes, research in the area is still in its infancy.
OBJECTIVE: This study investigated the role of psychological factors relating to symptom severity and quality of life, and differences between Raynaud\'s types (primary and secondary) to further inform the development of intervention in this field.
METHODS: A cross-sectional design was used. Two hundred and ten adults with Raynaud\'s completed an online questionnaire measuring stress, anxiety, depression, anxiety sensitivity, beliefs about emotions, symptom severity and quality of life.
RESULTS: Primary and secondary Raynaud\'s groups differed in anxiety (p < .004), symptom severity (p < .001) and quality of life (p < .001). Stepwise multiple regressions indicated anxiety and Raynaud\'s type explained 23% variance in hand symptom severity (p < .001); anxiety, Raynaud\'s type and anxiety sensitivity explained 29% variance in symptom severity (global impact, p < .001); depression, Raynaud\'s type and anxiety sensitivity explained 32% variance in quality of life (p < .001).
CONCLUSIONS: Results highlight the importance of psychological factors in Raynaud\'s phenomenon, indicating possible targets for treatment. Interventions such as cognitive behavioural therapy, which target both physical and psychological wellbeing, bear some promise as an adjuvant therapy for this group.

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