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.

Association of Pulmonary Hypertension and Monoclonal Gammopathy of Undetermined Significance

“…Autologous stern cell transplantations were performed in both patients, and the pulmonary hypertension resolved after complete remission was achieved [9]. Tere have been other case reports as well describing a reversible form of precapillary PH associated with MM [10,11,27]. A recent retrospective study of 359 patients with MM found that of the 123 patients who underwent echocardiography, 39 (32%) had elevated RVSP suggesting PH [28].…”

“…In fact, PH is a known pulmonary manifestation of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome, present in 27-48% of POEMS patients, often with resolution of elevated pulmonary pressures after treatment [6][7][8]. Association of PH with multiple myeloma (MM) [9][10][11][12] and amyloidosis have been reported [13].…”

Dermato-neuro Syndrome after COVID-19 Vaccination in a Patient with Scleromyxoedema Previously Controlled with Bortezomib and Intravenous Immunoglobulins

“…It is commonly used to treat relapsed/refractory multiple myeloma. Its use in SMX has been reported in 8 patients who differed in terms of clinical history, presentation and outcome, as well as treatment regimens (4)(5)(6)(7)(8)(9)(10)(11)(12)(13).…”

Pulmonary hypertension in patients with multiple myeloma: A comprehensive review

“…In most reports, PH‐MM‐TE related to drug‐induced PAH was reversible while in others both PH and MM were progressive and irreversible. 68 , 72 , 73 , 74 , 75 , 76 , 77 Feyereisn et al also report a case of likely, PH‐MM‐TE treated with Epoprostenol and calcium channel blocker in severe PAH that is vasodilator responsive. 71 Krishnan and colleagues report improvement in functional class and symptoms after institution of PAH treatment with sildenafil and ambrisentan in 3 patients PH‐MM‐TE that developed years after SCT.…”

“…In most instances of PH‐MM‐ND, the treatment of MM might improve PH and therefore, might be the preferred way in patients with minimal PH and WHO FC‐I and II patients. 8 , 67 , 68 If possible, the selection of chemotherapy regimen should aim to minimize further “hits” on the cardiovascular system and pulmonary circulation. There is emerging literature that adding metformin or angiotension II receptor blockers might be cardioprotective in patients receiving carfilzomib.…”