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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.
RESUMO
OBJECTIVES: To assess the levels of state and trait anxiety and determine their relationships with perceived social support among Nigerian patients with rheumatoid arthritis (RA). MATERIAL AND METHODS: A cross-sectional study of 50 patients satisfying the 2010 American College of Rheumatology/European League against Rheumatism Classification Criteria for RA was conducted. Anxiety was assessed using the Spielberger State-Trait Anxiety Inventory (STAI), perceived social support by the Interpersonal Support Evaluation List (ISEL), health-related quality of life (HRQoL) by the Medical Outcome Study 36-Item Short Form Health Survey (SF-36) and disability by the Health Assessment Questionnaire-Disability Index (HAQ-DI). RESULTS: The mean state anxiety (STAI-S), trait anxiety (STAI-T) and ISEL scores among the patients were 35.2 ±10.2, 36.7 ±8.8 and 87.2 ±21.2 respectively. Pathological degrees of state and trait anxiety were found among 7 (14%) and 5 (10.4%) patients respectively. There was a negative correlation between the STAI-T score and the ISEL score (r = -0.362, p = 0.011). However, the correlation between STAI-S and ISEL was not statistically significant (r = -0.193, p = 0.179). A moderate-to-high correlation was found between each of STAI-S and STAI-T and all subscales and component summaries of the SF-36. ISEL score correlated significantly with role emotional (r = 0.377, p = 0.008), mental health (r = 0.482, p ≤ 0.001) and bodily pain (r = 0.320, p = 0.025) domains and the mental component summary (r = 0.380, p = 0.007) of SF-36. HAQ-DI correlated strongly with both STAI-S (r = 0.735, p ≤ 0.001) and STAI-T (r = 0.575, p ≤ 0.001) but not with ISEL. CONCLUSIONS: State and trait anxiety correlate negatively with all aspects of HRQoL and disability, and there is a notable relationship between perceived social support and trait anxiety as well as the mental aspect of HRQoL.
