AB0981 Clinical features of 28 cases of limb restricted vasculitis and fasciitis

Abstract

BackgroundWe sometimes experience the cases with fever and muscle pain of lower limbs without any other specific features. There are sporadic case reports of eosinophilic fasciitis and limb restricted vasculitis. However, few reports compare and discuss such cases.ObjectivesTo describe the clinical features, MRI findings, histopathology, diagnosis and response to treatment of these cases.MethodsWe retrospectively analyzed the clinical features of 28 patients who were admitted to our hospital because of fever and muscle pain of lower limbs from 2004 to 2016.ResultsAmong the 28 patients, 17 were vasculitis syndrome; eleven were limb restricted small vessel vasculitis (LrSvv), six were microscopic polyangiitis (MPA). Seven were fasciitis; three were eosinophilic fasciitis, three were diffuse fasciitis without eosinophilia and one was tuberculous fasciitis. One was relapsing polychondritis, one was Behçet's disease and the other two were myalgia without specific diagnosis. In our study, average age was 57.5±19.9 years old and older than in previously reported cases of limb ristricted vasculitis1,2. Sixteen were female, twelve were male. Abnormal MRI findings in non-infectious fasciitis and vasculitis syndrome were bilateral. Tuberculous fasciitis showed specifically abnormal intensity and fluid collection in unilateral thigh. Unilateral lesion and fluid collection may indicate infectious disease and bilateral lesion may indicate autoimmune or autoinflammatory diseases. MRI of vasculitis syndrome and fasciitis showed hyperintense T2-weighted signals in muscles of either legs, or thighs, or both. (n=3), MPA (n=1) and fasciitis (n=3). On MRI scan, abnormal fascial signal intensity was seen in all the patients with fasciitis and 6 (40%) with vasculitis syndrome. It was difficult to differentiate between vasculitis and fasciitis by MRI findings. Muscle biopsy was performed in 25 patients. In most cases, we performed en bloc biopsy, including muscle, fascia, skin and subcutaneous tissue. MRI was useful to determine the location of biopsy. All patients were treated with glucocorticoids. Immunosuppressive agents (azathioprine, n=10; methotrexate, n=5; cyclophosphamide, n=1; tacrolimus, n=1) were added in 15 patients and anti-tuberculous drugs in one. None of the 11 patients with LrSvv showed positive blood tests of anti-neutrophil cytoplasmic antibody or developed any other organ involvement during follow-up period (median 96 months; range 3–125). They responded well to glucocorticoid therapy (oral prednisolone 0.5–0.6mg/kg/day or intravenous methylprednisolone at doses of 1g/day). Recurrence rate of LrSvv patients was 0%, although that of MPA patients was 50% (n=3). In four patients with LrSvv, treatment was ceased and they achieved drug-free remission. There were no apparent differences between the patients who achieved drug-free remission and who didn't.ConclusionsMRI and muscle biopsy were useful for diagnosis of disease with fever and muscle pain of lower limbs.References Gallien S, et al. Ann Rheum Dis 2002;61:1107–9.Kh...