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BMC Pulm Med. 2020 Jan 9;20(1):8. doi: 10.1186/s12890-019-1020-6.

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

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Department of Pulmonary and Critical Care Medicine, Marshfield Clinic, Marshfield, WI, USA.
Present affiliation: Christiana Care Health System, PO Box 1668, Wilmington, DE, 19899, USA.
Department of Internal Medicine, Marshfield Clinic, Marshfield, WI, USA.
Department of Rheumatology, Ronald Reagan UCLA Medical Center, Santa Monica, California, USA.
Department of Pathology, Marshfield Clinic, Marshfield, WI, USA.



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.


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.


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.


Bortezomib; Cyclophosphamide; Dexamethasone; Multiple myeloma; Pulmonary hypertension; Scleromyxedema

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