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

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Mazen Kreidy ◽  
Ali Al-Hilli ◽  
Ralph Yachoui ◽  
Jeffrey Resnick
Keyword(s):  
Multiple Myeloma ◽  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Diuretic Therapy ◽  
Pulmonary Involvement ◽  
Skin Lesions ◽  
Underlying Disease ◽  
Connective Tissue Disorder ◽  
Plasma Cell Dyscrasia ◽  
Cell Counts

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.

scholarly journals Pulmonary involvement in systemic sclerosis due to therapy and as a complication

2005 ◽  
Vol 63 (2) ◽  
Author(s):  
F. Chiappini ◽  
A. Zoli ◽  
L. Maugeri
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Lung Disease ◽  
Side Effect ◽  
Connective Tissue Diseases ◽  
Pulmonary Involvement ◽  
Clinical Conditions

(SS), similar to other connective tissue diseases. Sometimes lung disease may be a side effect of antirheumathic drugs. We report a case of a patient affected by SS, with isolated pulmonary hypertension, who developed bronchiolitis secondary to penicillamine. The latter was treated by withdraw of the drug without increasing the steroids. After one month, the patient’s clinical conditions appeared improved.

scholarly journals Echocardiographic Prevalence and Risk Predictors of Ventricular Dysfunction in Connective Tissue Disorders: Tertiary Care Hospital-Based Prospective Case-Control Study

2020 ◽  
Vol 1 (3-4) ◽  
pp. 132-141
Author(s):  
Saru Thakur ◽  
Geeta Ram Tegta ◽  
Prakash Chand Negi ◽  
Kunal Mahajan ◽  
Ghanshyam Verma ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Systolic Dysfunction ◽  
Tertiary Care ◽  
Left Ventricular ◽  
Care Hospital ◽  
Connective Tissue Disorders ◽  
Cardiac Symptom ◽  
Recorded Data

Background: There is a paucity of contemporary Indian data about the prevalence of cardiac abnormalities in patients of connective tissue disorders (CTD) and their risk determinants. Methods: We prospectively recorded data from 35 consecutive CTD patients who presented to our out-patient department and had no significant cardiovascular risk factors at baseline. We also recorded data from their age- and sex-matched controls. All cases and controls were subjected to 12 lead electrocardiogram and echocardiography after routine investigations. Results: The CTD group comprised 19 (54.3%) patients of systemic lupus erythematosus, 12 (34.3%) patients of systemic sclerosis, 2 (5.7%) patients of mixed CTD, and 1 (2.9%) patient each of overlap syndrome and dermatomyositis. Cardiovascular involvement on echocardiography was documented in 71.4% of CTD patients despite majority of them having no cardiac symptom. Overt left ventricular (LV) systolic dysfunction was observed in 3 (8.6%) CTD patients, while subclinical LV systolic dysfunction was recorded in 13 (37.1%) patients. LV diastolic dysfunction was observed in 11.4% (n = 4) patients. RV systolic dysfunction was prevalent in 20% (n = 7) patients. Pulmonary hypertension was observed in 40% (n = 14) of CTD patients. Conclusion: The present study evaluated subclinical LV systolic dysfunction and pulmonary hypertension in about one third of CTD patients. It is imperative to screen for these abnormalities in CTD to ensure timely diagnosis and treatment.

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