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Severe aortic regurgitation in RF positive polyarticular JIA
  1. I E M Bultink1,
  2. W F Lems1,
  3. B A C Dijkmans1,
  4. R M van Soesbergen1,
  5. J Lindeman2
  1. 1Department of Rheumatology, Slotervaart Hospital, Amsterdam, Jan van Breemen Institute, Amsterdam, and Vrije Universiteit Medical Centre, Amsterdam, The Netherlands
  2. 2Department of Pathology, Slotervaart Hospital, Amsterdam, The Netherlands
  1. Correspondence to:
    Dr I E M Bultink, Department of Rheumatology, Slotervaart Hospital, Louwesweg 6 1066 EC, Amsterdam, The Netherlands;
    iem_bultink{at}hotmail.com

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An 18 year old girl of Moroccan origin with a clear medical history was transferred to the Netherlands in February 1989 because of a two year history of untreated polyarthritis. The disease had pursued a rapidly destructive course, resulting in contractures and ankylosis of hips, knees, shoulders, and elbows and small joint deformation. A diagnosis of juvenile idiopathic arthritis (JIA) polyarticular type, functional class IV was made. No nodules were present. Laboratory analysis at that time showed borderline positive serum rheumatoid factor (RF) 30 IE/ml. Tests for antinuclear antibodies and HLA-B27 were negative. Treatment was started with intensive physiotherapy and intramuscular gold, the latter being replaced by sulfasalazine because of proteinuria. In 1990 she was treated for a unilateral uveitis. In 1992 her right elbow was replaced. Until 1993 cardiac examination showed no murmurs and chest roentgenogram was normal.

In November 1995 she was admitted because of a six month history of progressive respiratory distress and increasingly frequent attacks of angina pectoris. Her heart rate was 84 beats/min with a blood pressure of 160/0 mm Hg. A grade 3/6 systolic ejection murmur that radiated into the ascending aorta was heard over the cardiac apex as well as a grade 3/6 early diastolic decrescendo murmur over the left sternal border. A pericardial friction rub was not present. Examination of the carotid arteries disclosed a murmur and palpable thrill on both sides. An electrocardiogram showed left ventricular hypertrophy and the chest radiograph slight cardiomegaly. An echocardiogram demonstrated left ventricular dilatation (65 mm; normally <55 mm) and an abnormally thickened aortic valve. Colour Doppler echocardiography showed severe aortic regurgitation, a pressure gradient over the aortic valve (maximum pressure gradient 38 mm Hg, mean gradient 24 mm Hg), and diastolic back flow in the abdominal aorta. The diagnosis aortic valve insufficiency and secondary angina pectoris was made.

She underwent surgical replacement of her aortic valve with a Medtronic Hall prosthetic valve No 21. The postoperative course was uneventful. Pathological evaluation of the excised strongly thickened and fibrotic trileaflet aortic valve was performed.

Microscopic findings in one of the rheumatoid leaflets showed granulation tissue with lymphoplasmocellular infiltration and some polymorphonuclear cells around two areas of fibrinoid necrosis surrounded by a palisade of histiocytes (figs 1 and 2). These findings are similar to the description of a developed typical rheumatoid nodule.1

At follow up after four years the aortic valve prosthesis still functions well and the patient has no cardial signs and symptoms .

To our knowledge, this case is the first illustrated report of typical rheumatoid nodules found in an aortic valve removed owing to aortic valve insufficiency in a patient with polyarticular JIA. Our patient never had any nodules on other locations. Valvular disease is rare in patients with JIA2 and consists of valvulitis with a substrate with non-specific changes of fibrosis and necrosis. Valvular involvement has been described in patients with all types of JIA,3 the aortic valve being most commonly affected.4–9 Valvular disease is associated with severe destructive articular disease.4–7

Furthermore, our case report confirms the possibility of successful mechanical aortic valve replacement in a case of severe progressive aortic valve insufficiency and secondary angina pectoris in a patient with polyarticular JIA.

We recommend regular cardiac appraisal as part of the routine assessment of every patient with JIA. Whenever cardiac murmurs are detected in these patients, echocardiographic assessment should be considered, because if there is valve insufficiency the cardiac function may deteriorate and cardiac surgery may be needed.

Figure 1

Section from aortic valve cusp showing a central area of fibrinoid necrosis (a), a palisade of radially arranged histiocytes (b), and a lymphoplasmocytic infiltrate (c) (haematoxylin and eosin). Bar represents 400 μm.

Figure 2

Detail of an area of fibrinoid necrosis surrounded by a palisade of histiocytes; infiltration predominantly with lymphocytes and plasma cells (haematoxylin and eosin). Bar represents 25 μm.

Acknowledgments

We are grateful to Dr J van der Meulen, cardiothoracic surgeon, for the surgical description and to Dr AC van der Wal, pathologist, for his pathology specimen evaluation. We thank Dr FM Westerweel, rheumatologist, for allowing us to report on her patient.

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