Rheumatology Advance Access originally published online on October 1, 2007
Rheumatology 2007 46(11):1737-1738; doi:10.1093/rheumatology/kem237
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A case of rheumatoid vasculitis involving the gastrointestinal tract in early disease
Department of Rheumatology, Lancashire Teaching Hospitals NHS Trust, Royal Preston Hospital, Sharoe Green Lane North, Preston PR2 9HT, UK
Correspondence to: Dr B. Parker, Department of Rheumatology, Lancashire Teaching Hospitals NHS Trust, Royal Preston Hospital, Sharoe Green Lane North, Preston PR2 9HT,UK. E-mail: ben.parker{at}lthtr.nhs.uk
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SIR, Rheumatoid vasculitis (RV) is a well-known complication of long-standing seropositive rheumatoid arthritis. We report an unusual case affecting multiple organs, including the gastrointestinal (GI) tract, and occurring within 12 months of diagnosis.
In May 2006, a previously well 63-yr-old male presented with an abrupt onset of symmetrical inflammatory polyarthritis affecting the small joints of his hands and feet as well as his knees. He was strongly positive for both rheumatoid factor and anti-cyclic citrullinated peptide antibody, but initial X-rays revealed no erosive changes. He was diagnosed with rheumatoid arthritis and rapid symptom control was achieved with intra-muscular and intra-articular steroids. He was immediately started on oral methotrexate (10 mg, titrated up to 20 mg) and oral hydroxychloroquine (200 mg bd).
Unfortunately the methotrexate was stopped in July 2006 due to abnormal liver function tests, and following a subsequent episode of colicky upper abdominal pain, an ultrasound scan revealed multiple gallstones. He was listed for cholecystectomy by the surgical team. Methotrexate was not restarted.
Prior to his cholecystectomy, he presented in January 2007 to the acute medical team with an abrupt onset of bilateral parasthesia to his lower legs (and to a lesser degree his hands), generalized weakness, fatigue and fevers. Clinically, he had nail fold infarcts and a symmetrical sensory peripheral neuropathy most prominent in his lower limbs. Musculoskeletal examination revealed low-grade inflammatory joint disease affecting his metacarpo-phalangeal joints. Systemic examination did not reveal any other abnormality.
Extensive further investigation excluded alternative diagnoses (such as subacute bacterial endocarditis, cryoglobulinaemia and lymphoma) and included blood film, urine dipstick, chest X-ray, multiple blood cultures, hepatitis screen, echocardiography and cardiology review. No specific request was made of the microbiology department to look for Haemophilus aphrophilus; Actinobacillus actinomycetemcomitans; Cardiobacterium hominis; Eikenella corrodens; and Kingella kingae organisms. A CT scan of his chest and abdomen performed during the course of his illness did not show any lymphadenopathy. ESR was 110 mm/h and full immunology screen was negative, apart from a positive rheumatoid factor.
Electrophysiological studies demonstrated bilateral carpal tunnel syndrome, an asymmetric polyneuropathy and mononeuropathies of both sciatic nerves, as well as an underlying inflammatory myositis.
Systemic RV was therefore diagnosed and he was treated with 30 mg oral prednisolone with improvement in his neuropathic symptoms noted within 48 h i.v. cyclophosphamide was initially planned but temporarily withheld after a further episode of colicky abdominal pain, which on this occasion was diagnosed as diverticulitis as repeat ultra-sound scan did not reveal gallstones. He received his first dose of i.v. cyclophosphamide in February 2007 and remained on oral steroids.
In March 2007, he presented to accident and emergency with severe abdominal pain, nausea and vomiting for 2 days. He underwent urgent exploratory laparotomy, where a small bowel perforation was identified. No alternative cause for these symptoms was found and no lymphadenopathy was noted. Histology revealed endarteritis obliterans of the medium-sized arteries with recanalization, suggestive of end-stage RV.
Based on the history, clinical examination, serology, electrophysiological studies and histology of the small bowel, a diagnosis of systemic RV affecting the peripheral nervous system, GI system and skeletal muscle was made. Extensive investigation failed to demonstrate an alternative diagnosis.
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RV is a systemic necrotizing vasculitis affecting small and medium-sized vessels. It commonly presents in established seropositive patients with inactive, destructive disease and the mean onset is 13 yrs after diagnosis [1]. It is considered rare in early disease [2, 3]. It is characterized histopathologically in the acute stages by fibrinoid necrosis with an inflammatory infiltrate, and in chronic cases by vessel wall fibrosis, occlusion and recanalization. Clinical features include Raynaud's phenomenon, nail fold and digital infarcts, skin ulceration, mononeuritis multiplex, scleromalacia perforans and occlusion of arteries to visceral organs. Non-specific features are common and include high inflammatory markers and constitutional symptoms such as fatigue, weight loss and fevers.
GI involvement is rare. There have been case reports of GI involvement, leading to intra-abdominal haemorrhage [4], GI infarction [5], and multiple intestinal ulcers [6], but these have either been in well-established disease or in association with other localized pathology such as amyloid A amyloidosis [4]. It is possible that some of the earlier reported cases of GI involvement actually represented other vasculitides, such as polyarteritis nodosa.
The peripheral nervous system is commonly involved in RV in the form of mononeuritis multiplex and peripheral neuropathy [7]. However, muscle disease is less commonly involved clinically (on testing and with serum creatine kinase) but appears to be involved subclinically in a large number of patients with other systemic features when electromyography is performed [8].
We believe this case is important for several reasons. First, and most importantly, it highlights that RV can complicate very early rheumatoid arthritis and that the presence of non-articular symptoms and constitutional symptoms should alert the physician to its possible presence. Secondly, although rare, the GI tract can be affected in RV. The presence of GI symptoms in a patient with other features of a systemic vasculitis (primary or secondary) should be investigated thoroughly, even if other causes may be present (in this case, the diagnoses of gallstones and diverticulitis were both made). Finally, with the advent of more aggressive treatment regimens and better control of early disease, RV may become less common. Those cases thus encountered may be of the more unusual manifestations and in early aggressive disease.
Disclosure statement: The authors have declared no conflicts of interest.
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- Maini RN, Warrell DA, Cox TM, Firth JD, Benz EJ. Oxford textbook of medicine, 4th edn. Rheumatoid arthritis. vol 3. Oxford University Press, 2003:33.
- Voskuyl AE, Zwinderman AH, Westedt ML, et al. Factors associated with the development of vasculitis in rheumatoid arthritis: results of a case-control study. Ann Rheum Dis (1996) 55:190.
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[Abstract/Free Full Text]
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