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

Abstract:

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.

journal_name

BMC Pulm Med

journal_title

BMC pulmonary medicine

authors

Kreidy M,Al-Hilli A,Yachoui R,Resnick J

doi

10.1186/s12890-019-1020-6

subject

Has Abstract

pub_date

2020-01-09 00:00:00

pages

8

issue

1

issn

1471-2466

pii

10.1186/s12890-019-1020-6

journal_volume

20

pub_type

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