Compression Stockings Improve Lower Legs Symptom in Patients with Pulmonary Artery Hypertension Treated by Pulmonary Vasodilators-A Pilot Study.

Pulmonary vasodilators have improved pulmonary arterial hypertension (PAH) symptoms and prognosis; however, the drugs cause some side effects, including lower legs pain, which impair quality of life (QOL). The present study examined if compression stockings improved lower extremity symptoms and QOL caused by pulmonary vasodilators in PAH patients. We retrospectively enrolled consecutively ten patients with PAH treated by pulmonary vasodilators, who were regularly followed in Kurume University Hospital from January 2022 to June 2022. Oral questionnaire surveys, the Numeric Rating Scale for Pain (NRS) and the Pain Disability Assessment Scale (PDAS), were conducted regarding lower extremity symptoms before wearing elastic stockings and one month later, to evaluate how the lower extremity symptoms affected daily life. All ten patients were female, with a mean age of 50.2 ± 11.5 years, out of whom intravenous prostacyclin analogue (PGI2) was administered in five patients. In no intravenous PGI2 group, NRS score was significantly improved from 4.6 ± 2.3 to 2.8 ± 1.2 (p = 0.037), while from 9.4 ± 1.2 to 5.4 ± 1.6 (p = 0.002) in intravenous PGI2 group. PDAS score was also significantly improved [no intravenous PGI2 group; 18.0 (15.0-24.0) to 15.0 (10.0-19.0), intravenous PGI2 group; 25.0 (17.0-37.0) to 17.0 (5.0-27.0)]. Lower extremity symptoms in patients using pulmonary vasodilators were improved by wearing compression stockings.

Digital gangrene in systemic lupus erythematosus.

Digital ulcers and gangrene are common skin manifestations of connective tissue diseases, especially systemic sclerosis, although they are relatively rare in systemic lupus erythematosus. We describe here three patients with digital gangrene and systemic lupus erythematosus. None of the patients showed high disease activity of systemic lupus erythematosus at the time the digital gangrene developed. Two patients were positive for anti-RNP antibodies; however, no symptoms of other collagen diseases were present. One patient had anti-phosphatidylserine/prothrombin complex antibodies, and the other had anti-cardiolipin beta2 glycoprotein I antibodies and lupus anticoagulant at low titre. All patients showed narrowing or occlusion of radial and/or ulnar arteries in addition to digital arteries. Although a complication of anti-phospholipid syndrome is considered to be a possible cause, there may be unidentified causes other than thrombosis, atherosclerosis, overlap syndrome and vasculitis.

Association of antiphosphatidylserine/prothrombin antibodies with neuropsychiatric systemic lupus erythematosus.

Neuropsychiatric manifestations in patients with systemic lupus erythematosus (SLE) are well-recognized symptoms although the pathophysiology of neuropsychiatric SLE (NPSLE) is unclear. Since an association with antiphospholipid antibodies has been reported, we examined the prevalence of antiphosphatidylserine-prothrombin antibodies (anti-PS/PT Abs), lupus anticoagulant (LA), anticardiolipin/beta2-glycoprotein I antibodies (anti-beta2-GPI Abs), and antiribosomal P protein antibodies (antiribosomal P Abs) in 68 SLE patients and analyzed their associations with neuropsychiatric manifestations. The prevalence of LA was significantly higher in the patients with neuropsychiatric (NP) features than those without NP features (P < 0.02). The levels of anti-PS/PT antibody were also significantly higher in the patients with NP features than those without NP features (P < 0.01). The results indicate that LA positivity and higher levels of anti-PS/PT antibody can be predictive markers for NPSLE.

Cutaneous manifestations and histological features of microscopic polyangiitis.

Purpura and livedo are common cutaneous manifestations of microscopic polyangiitis (MPA); however, only a few clinical analyses focusing on the relationship between clinical symptoms and the affected vessels in the skin have been reported. We herein report the cutaneous manifestations and histological features of four patients with MPA. In two patients, a Henoch-Shönlein purpura-like eruption developed with necrotizing vasculitis localized in the upper dermis. The other two patients presented with livedo racemosa; with histological findings of necrotizing vasculitis of the small vessels around the muscle fibers or from the deep dermis to subcutaneous tissue. Two patients' cases were complicated by systemic sclerosis and had poor prognoses. MPA can present with various cutaneous manifestations. Additional research is required to ascertain the relationship between the prognosis and the affected vessels.