Severe but reversible pulmonary hypertension in scleromyxedema and multiple myeloma: a case report

Mazen Kreidy,Ali Al-Hilli,Ralph Yachoui,Jeffrey Resnick

doi:10.1186/s12890-019-1020-6

Abstract

BackgroundScleromyxedema 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 presentationAn 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.ConclusionsTreatment 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.

Highlights

Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels
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
Scleromyxedema is a rare, chronic, progressive disorder characterized by skin lesions with mucinous material deposition, fibrosis, increased population of fibroblasts, and high levels of immunoglobulins in serum without a corresponding thyroid abnormality [1,2,3,4,5,6,7,8,9,10,11]

Summary

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.

Background

Discussion

Findings

Conclusion