BACKGROUND Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels. Pulmonary involvement occurs in a subset of patients, though the overall prevalence of pulmonary lesions in scleromyxedema is unknown. Since pulmonary hypertension presumably occurs in these patients due to disease progression and development of additional conditions, treatment of the underlying plasma cell dyscrasia and connective tissue disorder may improve pulmonary hypertension symptoms. CASE PRESENTATION An elderly patient with scleromyxedema developed pulmonary hypertension refractory to vasodilator and diuretic therapy and subsequently multiple myeloma that responded to a combination therapy of bortezomib, cyclophosphamide, and dexamethasone treatment. CONCLUSIONS Treatment of the underlying disease(s) that contributed to pulmonary hypertension development with anti-neoplastic agents like bortezomib may improve cardiopulmonary symptoms secondary to reducing abnormal blood cell counts and paraprotein levels.

中文翻译：

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硬化粘液水肿和多发性骨髓瘤中的严重但可逆性肺动脉高压：病例报告。


背景硬化性粘液水肿是一种进行性、全身性结缔组织疾病，其特征在于纤维粘液皮肤病变和血清单克隆免疫球蛋白水平升高。尽管硬化粘液水肿中肺部病变的总体患病率尚不清楚，但一小部分患者会出现肺部受累。由于这些患者可能由于疾病进展和其他病症的发展而发生肺动脉高压，因此治疗潜在的浆细胞恶液质和结缔组织疾病可能会改善肺动脉高压症状。病例介绍 一名患有硬化性粘液水肿的老年患者出现了对血管扩张剂和利尿剂治疗无效的肺动脉高压，随后出现了对硼替佐米、环磷酰胺和地塞米松联合治疗有反应的多发性骨髓瘤。结论 使用硼替佐米等抗肿瘤药物治疗导致肺动脉高压发展的基础疾病，可以改善继发于减少异常血细胞计数和副蛋白水平的心肺症状。