28202869 Visualization

A 34 Age - year Age - old Age Japanese Personal_background woman Sex had a diagnosis of MCTD Disease_disorder with the presence of Raynaud's Disease_disorder phenomenon Disease_disorder , pancytopenia Sign_symptom , elevated Lab_value plasma Detailed_description creatinine Diagnostic_procedure kinase Diagnostic_procedure , and antibodies Lab_value against Lab_value U1 Diagnostic_procedure - ribonucleoprotein Diagnostic_procedure in 2005 Date and received 10 Dosage mg Dosage of prednisolone Medication daily Dosage .
Pulmonary Diagnostic_procedure arterial Diagnostic_procedure systolic Diagnostic_procedure pressure Diagnostic_procedure estimated by echocardiography Diagnostic_procedure was slightly Lab_value elevated Lab_value ( 48 Lab_value mmHg Lab_value ) in 2006 Date .
She felt progressive Detailed_description shortness Sign_symptom of Sign_symptom breath Sign_symptom on physical Activity effort Activity in 2011 Date .
Pulmonary Disease_disorder arterial Disease_disorder hypertension Disease_disorder was diagnosed with 74 Lab_value mmHg Lab_value of mean Diagnostic_procedure pulmonary Diagnostic_procedure arterial Diagnostic_procedure pressure Diagnostic_procedure ( mPAP Diagnostic_procedure ) evaluated by right Diagnostic_procedure heart Diagnostic_procedure catheterization Diagnostic_procedure ( RHC Diagnostic_procedure ) in 2012 Date .
Cyclophosphamide Medication -based immunosuppressive Medication therapy Medication was proposed but refused because of possible early Detailed_description menopausal Sign_symptom symptoms Sign_symptom .
Treatment with 250 Dosage mg/day Dosage of bosentan Medication and 120 Dosage μg/day Dosage of beraprost Medication was initiated.
However, in January Date 2014 Date , her dyspnea Sign_symptom deteriorated ( WHO Diagnostic_procedure functional Diagnostic_procedure class Diagnostic_procedure IV Lab_value ), and she was admitted Clinical_event to our hospital Nonbiological_location .
A physical Diagnostic_procedure examination Diagnostic_procedure showed low Lab_value systemic Diagnostic_procedure blood Diagnostic_procedure pressure Diagnostic_procedure ( 89/61 Lab_value mmHg Lab_value ), tachycardia Sign_symptom ( 104/min Lab_value ), low Lab_value blood Diagnostic_procedure oxygen Diagnostic_procedure level Diagnostic_procedure ( percutaneous Diagnostic_procedure oxygen Diagnostic_procedure saturation Diagnostic_procedure ( SpO2 Diagnostic_procedure ) 95% Lab_value ), jugular Biological_structure venous Biological_structure distension Sign_symptom , and severe Severity systemic Detailed_description edema Sign_symptom .
Coarse Detailed_description crackles Sign_symptom and loud Detailed_description pulmonic Sign_symptom valve Sign_symptom closure Sign_symptom sounds Sign_symptom were detected.
She was unable to undergo the 6 Diagnostic_procedure - minute Diagnostic_procedure walk Diagnostic_procedure test Diagnostic_procedure due to severe Severity dyspnea Sign_symptom .
Laboratory Diagnostic_procedure examinations Diagnostic_procedure showed elevated Lab_value plasma Detailed_description brain Diagnostic_procedure natriuretic Diagnostic_procedure peptide Diagnostic_procedure ( BNP Diagnostic_procedure ) level ( 929.4 Lab_value pg/mL Lab_value ), hypoxemia Sign_symptom ( PaO2 Diagnostic_procedure 62.5 Lab_value mmHg Lab_value , PaCO2 Diagnostic_procedure 27.1 Lab_value mmHg Lab_value ), and the presence Lab_value of Lab_value antibodies Lab_value against Lab_value U1 Diagnostic_procedure - ribonucleoprotein Diagnostic_procedure ( 97.9 Lab_value U/mL Lab_value ) as well as antinuclear Diagnostic_procedure antibodies Diagnostic_procedure ( 1:640 Lab_value , speckled Lab_value pattern Lab_value ).
A chest Biological_structure X Diagnostic_procedure - ray Diagnostic_procedure showed enlarged Sign_symptom pulmonary Biological_structure arteries Biological_structure and cardiomegaly Sign_symptom (Figure).
Electrocardiogram Diagnostic_procedure showed P Sign_symptom wave Sign_symptom elevation Sign_symptom in the V1 Detailed_description - V2 Detailed_description leads Detailed_description .
RHC Diagnostic_procedure demonstrated elevated Lab_value mPAP Diagnostic_procedure ( 65 Lab_value mmHg Lab_value ) with normal Lab_value pulmonary Diagnostic_procedure arterial Diagnostic_procedure wedge Diagnostic_procedure pressure Diagnostic_procedure ( 12 Lab_value mmHg Lab_value ), high Lab_value pulmonary Diagnostic_procedure vascular Diagnostic_procedure resistance Diagnostic_procedure ( 1,547 Lab_value dyne・sec・cm-5), and decreased Lab_value cardiac Diagnostic_procedure output Diagnostic_procedure ( cardiac Lab_value index Lab_value 1.69 Lab_value L/min/m2 Lab_value ).
A ventilation/perfusion Diagnostic_procedure scan Diagnostic_procedure showed no signs of pulmonary Disease_disorder thromboembolism Disease_disorder .
No signs of chronic Disease_disorder obstructive Disease_disorder pulmonary Disease_disorder disease Disease_disorder or interstitial Disease_disorder lung Disease_disorder diseases Disease_disorder were found with computed Diagnostic_procedure tomography Diagnostic_procedure .
Her dyspnea Sign_symptom was therefore considered to be due to MCTD Disease_disorder -associated PAH Disease_disorder which was refractory to bosentan Medication and beraprost Medication therapy.
In addition to supportive Therapeutic_procedure therapy Therapeutic_procedure with oxygen Therapeutic_procedure supplementation Therapeutic_procedure and diuretics Medication ( 40 Dosage mg/day Dosage of furosemide Medication ), initiation of 60 Dosage mg/day Dosage of sildenafil Medication as well as gradual Dosage increment Dosage in Dosage the Dosage dose Dosage of beraprost Medication was chosen as an additional treatment.
However, her dyspnea Sign_symptom remained unchanged, and her plasma Detailed_description BNP Diagnostic_procedure level increased Lab_value .
IVCY Medication ( 750 Dosage mg/m2, Dosage every Dosage 4 Dosage weeks Dosage ) was initiated, and the dose of prednisolone Medication was increased to 60 Dosage mg Dosage daily Dosage .
Her dyspnea Sign_symptom then ameliorated, and she became able to undergo the 6 Diagnostic_procedure - minute Diagnostic_procedure walk Diagnostic_procedure test Diagnostic_procedure ( 260 Lab_value m Lab_value , minimum Diagnostic_procedure SpO2 Diagnostic_procedure 95 Lab_value % Lab_value ).
Her plasma Detailed_description BNP Diagnostic_procedure level also decreased Lab_value after the initiation of IVCY Medication (Figure).
Six Date months Date after Date the admission Clinical_event , a follow-up RHC Diagnostic_procedure revealed improved Lab_value mPAP Diagnostic_procedure (65 to 53 Lab_value mmHg Lab_value ), pulmonary Diagnostic_procedure vascular Diagnostic_procedure resistance Diagnostic_procedure (1,547 to 1,116 Lab_value dyne・sec・cm-5), and cardiac Diagnostic_procedure index Diagnostic_procedure (1.69 to 1.83 Lab_value L/min/m2 Lab_value ) (Table).
Since she remained stable Sign_symptom but did not obtain further improvements 18 Date months Date after Date the admission Clinical_event (Table), we consider parenteral Administration prostanoids Medication or lung Therapeutic_procedure transplantation Therapeutic_procedure as a subsequent treatment.