Department of Medicine, School of Medicine and 1 School of Medical Technology, Chung Shan Medical University and 2 Division of Allergy, Immunology and Rheumatology, Department of Medicine, Chung Shan Medical University Hospital, Taiwan
Correspondence to: J. C.-C. Wei, Division of Allergy, Immunology and Rheumatology, Department of Medicine, Chung Shan Medical University Hospital, No. 110, Sec. 1, Jianguo N. Road, South District, Taichung City 40201, Taiwan. E-mail: wei3228{at}ms3.hinet.net
SIR, We report the case of a 58-yr-old female who complained of increasing tightness and tingling pain in the hands and face for several weeks. The patient had been well until 3 months previously when she developed flu-like symptoms followed by fatigue, fever and puffiness of the hands. She also noted that blanching with subsequent cyanosis and redness of fingers became evident on exposure to cold. In addition, dry eyes and mouth were also recognized.
One week later, she developed bilateral leg oedema and low back pain along with arthralgia of the knees and ankles. She also felt that the skin over her neck and shoulders became tighter and thicker. The possibility of progressive systemic sclerosis (PSS) was raised and she was admitted for further evaluation.
The family history revealed that her second son had Reiter's syndrome and her third son had ankylosing spondylitis. Both sons were positive for human leucocyte antigen (HLA) B27 molecule.
The important laboratory findings were as follows: erythrocyte sedimentation rate (ESR) 17 mm/h, antinuclear antibodies (ANA) titre 1:1280 (speckled and nucleolar pattern), complement C3 51.5 mg/dl (normal 90180 mg/dl), complement C4 <5.42 mg/dl (normal 1040 mg/dl), HLA-B27 positive. Tests for haemoglobin, leucocyte count, C-reactive protein, anti-extractable nuclear antigen (ENA) antibodies, anticardiolipin antibodies, lupus anticoagulants, rheumatoid factor and cytoplasmic and perinuclear types of antineutrophil cytoplasmic antibodies (ANCA) were all normal or negative. IgM and IgG antibodies to parvovirus B19 and EpsteinBarr viral capsid antigen were all positive.
Imaging studies showed mild right sacroiliitis and C36 herniated intervertebral discs. Lesional skin biopsy of the wrist was performed for histology and characterization of B19 virus by DNA in situ hybridization was positive. The microscopic appearance of the sclerotic skin lesion is shown in Fig. 1.
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PSS is a complicated autoimmune disease whose aetiology remains unknown and there is increasing evidence implicating the involvement of infectious agents, such as parvovirus B19 [1, 2], in its pathogenesis. We present here a patient with scleroderma and possible concurrent infection with parvovirus B19 and EpsteinBarr virus (EBV). Active parvovirus B19 and EBV infection were documented by positive IgM in serum by enzyme-linked immunosorbent assay and in situ hybridization in skin biopsy. On reviewing the literature, parvovirus B19 is shown to be able to establish latency in the bone marrow [3], from where the virus could spread to target tissues, and significant elevation of the virus DNA in the skin of systemic sclerosis patients has been proved [4]. To assume a cause and effect relationship between parvovirus B19 and scleroderma is an attractive concept; however, the demonstration of the viral DNA could be due to non-specific immune stimulation.
In the absence of any recognized effective treatment for scleroderma, we tried alefacept, the recombinant human lymphocyte function-associated antigen-3 (LFA-3) IgG1 fusion protein, which can inhibit T-lymphocyte activation via blockade of LFA-3 and CD2 co-stimulation between memory effector T cells and antigen-presenting cells. Alefacept was approved by the US Food and Drug Administration for chronic plaque psoriasis [5], but there are no reports of its use in scleroderma patients. However, the scleroderma symptoms persisted without conspicuous alleviation, suggesting that the LFA-3-CD2 signalling pathway may play little or no role in the immunopathogenesis of scleroderma.
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The authors have declared no conflicts of interest.
References
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