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The greatest challenge in diagnosing vasculitis is the diversity of its clinical presentation. Awareness of the heterogeneity of uncommon manifestations can be decisive for the course of the disease.
We report on a patient presenting with periostitis as the initial manifestation of systemic vasculitis. A 38 year old female patient complained about progressively painful swelling and reddening of the distal right lower leg for several weeks. The patient had been healthy until then and had no history of arterial or venous insufficiency. She presented at hospital with reduced pulses and a severe compartment syndrome of the tibialis anterior compartment. An x ray examination showed typical signs of periostitis with periostal new bone formation (figs 1A and B).
Fasciotomy was done instantly and a periostal biopsy specimen was taken. Histopathological examination disclosed necrotising arteritis of small and medium sized arteries with polymorph neutrophilic infiltration of all layers of the vascular wall. Further investigation showed signs of systemic inflammation with a raised erythrocyte sedimentation rate (30 mm/1st h), C reactive protein (30 mg/l), and leucocytosis (12×109/l). Other serological markers were negative, likewise c- and pANCA and ANCA enzyme linked immunosorbent assay (ELISA). Hepatitis B and C were excluded. An apparently recent complete and singular occlusion of the A. tibialis anterior was demonstrated by angiography. Immunosuppressive treatment with methotrexate (10 mg/week, po) and oral prednisolone (10 mg/day) was started.
The patient was consecutively referred to our department because of recurrent painful swelling of the right lower leg and the development of scleritis, arthritis, and sensory peripheral neuropathy. Additionally, several other symptomatic arterial stenoses of the major aortic branches (A. subclavia, A. vertebralis, A. femoralis) were detected by angiography. According to the nomenclature of the Chapel Hill Consensus Conference, the patient’s disease was diagnosed as polyarteritis nodosa.1 Treatment was switched to cyclophosphamide (the so-called “NIH standard”: cyclophosphamide 2.0 mg/kg body weight per day with daily prednisolone po2). After induction of remission, treatment was switched to azathioprine. Follow up bone radiography disclosed a moderate reduction of the new periostal bone formation and clinical remission was maintained at a 3 year follow up.
The patient presented initially with an unusual manifestation of systemic vasculitis. Vasculitis restricted to the local vascular region may be the initial manifestation of systemic vasculitis. In this particular case, vasculitis of the periosteum might have induced local hypoxia of the bone, with subsequent release of bone derived growth factors and manifestation of periostitis.3 Periostitis is seen in many other conditions but is not common in necrotising vasculitis. It was described for the first time by Lovell and Scott in 1956.4 Until now only a few cases of periostitis in patients with polyarteritis nodosa have been reported; remarkably, the lower extremities were affected in all cases.5–7 However, periostitis has also been reported in other forms of systemic vasculitis.8,9,10 Most cases responded well to glucocorticoids. In refractory cases other cytotoxic treatment like methotrexate, azathioprine, or cyclophosphamide may be useful.
Thus, as demonstrated by this case, in patients with painful swelling of the lower limb, clinicians should consider periostitis as an unusual manifestation of systemic vasculitis.