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Mixed connective tissue disease complicated by heart failure in Ile-Ife, Nigeria: management challenges in a resource-limited economy

Authors Adewuya OA, Adebayo RA, Ajibade AI, Odunlami GJ, Akintomide AO, Ogunyemi SA, Ajayi OE, Adetiloye AO, Omisore AD, Olanipekun OA, Owolabi AO, Amjo I, Akinyele OA, Bamgboje AO, Balogun MO

Received 14 September 2017

Accepted for publication 22 June 2018

Published 2 November 2018 Volume 2018:11 Pages 307—312


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Ronald Prineas

Video abstract presented by Oladapo A Adewuya.

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Oladapo A Adewuya,1 Rasaaq A Adebayo,1 Adeola I Ajibade,2 Gbenga J Odunlami,2 Anthony O Akintomide,1 Suraj A Ogunyemi,1 Olufemi E Ajayi,1 Adebola O Adetiloye,3 Adeleye D Omisore,4 Oladipo A Olanipekun,1 Adeyinka O Owolabi,1 Ifeoluwa Amjo,1 Olumide A Akinyele,1 Abayomi O Bamgboje,1 Michael O Balogun1

1Department of Cardiology, 2Department of Rheumatology, 3Department of Pulmonology, 4Department of Radiology, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun, Nigeria

Background: Mixed connective tissue disease (MCTD; also known as Sharp’s syndrome) is a rare autoimmune inflammatory disorder characterized by high titer of U1 ribonucleoprotein (U1RNP) antibody and clinical and serological overlap of systemic lupus erythematosus, systemic sclerosis, and polymyositis. The diagnosis is based on clinical and serological factors in criteria such as Alarcon-Segovia, Khan, Kusakawa, and Sharps. Cardiac disease can be a complication of connective tissue disease (CTD). There are few reports in Africa.
Aims: To present MCTD as underlying cause of heart failure with reduced ejection fraction and highlight challenges of investigations and treatment.
Objectives: To highlight the first case in our center and discuss the cardiac, respiratory, and rheumatologic management.
Patient and methods: We present a 52-year-old woman with 3 weeks history of productive cough with whitish sputum, severe dyspnea, orthopnea, paroxysmal nocturnal dyspnea, right sided abdominal pain, leg swellings, a one year history of recurrent fever, Raynaud’s phenomenon, small joint swellings and deformities with pain in both hands.
Results: On examination there was microstomia, tethered forehead and lower eyelid skin, tender swelling of the interphalangeal joints and arthritis mutilans. Laboratory findings showed estimated glomerular filtration rate <60 mL/kg/min/1.73 m2, U1RNP antibody levels were eight times upper limit of normal, elevated rheumatoid factor, speckled antinuclear antibody pattern, negative anticentromere antibody, anti Scl-70 and anticyclic citrullinated peptide. Chest X-ray/CT revealed pulmonary fibrosis. Echocardiography findings showed reduced ejection fraction of 40%, elevated pulmonary arterial pressure at rest of 60.16 mmHg. The patient showed improvement on antifailure drugs, but prednisolone was stopped for sudden reversal of previously controlled stage 2 hypertension (HTN), and the patient was discharged in a stable condition. Difficulties ensued in obtaining prompt definite results due to the unavailability of serologic tests in the hospital, and the tests were done outside the state and country.
Conclusion: Identifying MCTD is critical, especially in patients requiring steroids that may worsen systemic HTN and heart failure. There is a need to have definitive investigative facilities for such patients in hospitals.

Keywords: mixed connective tissue disorder, U1RNP, arthritis mutilans PAH, HFrEF, prednisolone

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