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摘要
A 55-year-old Afro-Caribbean woman presented to the hospital with recent weight loss, generalized pruritus, malaise and shortness of breath caused by minimal exertion which progressed over recent months. Six months before admission she had attended the hospital with chest pain and acute shortness of breath. Cardiac enzyme levels were elevated, but the patient declined coronary angiography. Echocardiogram carried out during the admission 6 months previously revealed a left ventricular ejection fraction of 55-60%. Additional past medical history included hypertension and gastro-oesophageal reflux for which she was treated with ramipril and ranitidine respectively. In the previous 2 years she had been reviewed with regards to progressive skin depigmentation and was given a diagnosis of vitiligo. On examination, she was had diffuse areas of depigmentation with perifollicular sparing affecting the arms and torso (Figure 1). Facial and truncal skin tightening was noted in addition to minimal sclerodactyly distally with relative sparing of the finger tips (Figure 2). Cardiovascular examination revealed a mitral pan-systolic murmur with associated palpable right ventricular heave. The jugular venous pressure was elevated and she had extensive lower limb peripheral oedema. Auscultation of the chest revealed fine bibasal inspiration crepitations. Abdominal examination demonstrated non-tender hepatomegaly. Initial investigations showed raised inflammatory markers: C-reactive protein (33 mg/ litre, normal range <0.6 mg/litre) and erythrocyte sedimentation rate (121 mm/hour, normal range 1-20 mm/hour). Troponin T levels were significantly elevated at 314 ng/litre (normal range 0-14 ng/litre). Autoimmune serology interestingly revealed a negative antinuclear antibody, although anti-Ro antibodies were present. All other auto-antibodies were negative. Repeat echocardiogram demonstrated severe mitral regurgitation and impaired systolic function with a left ventricular ejection fraction of <20%. Subsequent investigation with cardiac magnetic resonance imaging showed chronic cardiomyopathy, secondary to the advanced scleroderma. The patient was placed on continuous cardiac monitoring, which noted a short run of non-sustained ventricular tachycardia. In the context of a negative anti-nuclear antibody, malignancy was excluded following extensive investigation, including serum tumour markers, positron emission tomography computed tomography, and bone marrow aspirate and trephine. Skin biopsy demonstrated thickened dermis composed of broad sclerotic collagen bundles and mucin deposition with perivascular inflammation, felt to be in keeping with systemic sclerosis. Atypical diffuse cutaneous systemic sclerosis with associated myocarditis was diagnosed. The patient was commenced on treatment with intravenous cyclophosphamide. Low dose oral prednisolone (10 mg/day), although controversial in the treatment of systemic sclerosis, was given in view of poor cardiac function secondary to myocarditis. In addition, bisoprolol, clopidogrel, ramipril, furosemide and spironolactone were started in view of worsening cardiac and renal function. Following the first dose of intravenous cyclophosphamide and low dose oral prednisolone, the patient clinically improved with resolution of the symptoms of cardiac failure. Cardiac enzymes progressively normalized. A rapid improvement was seen in the patient's skin with marked improvements in the degree of thickening, restriction and pigmentation. Unfortunately 2 months following discharge the patient died unexpectedly. The cause of death was thought to be an underlying cardiac arrhythmia secondary to chronic myocarditis.
- 出版日期2017-10
