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October 2014Patients with diffuse systemic sclerosis (dSS) with an associated ANCA-positive vasculitis have been infrequently described. In this article, we report the case of a 53-year-old woman, previously diagnosed with dSS, who developed an ANCA-associated vasculitis, which manifest as peripheral nerve and muscle disease.
Scleroderma is a disease characterized by sclerosis of the skin and visceral organs, vasculopathy, Raynaud’s phenomenon and the presence of autoantibodies. The disease spectrum is wide, with systemic and localized forms, depending on the extent of skin thickening. There is also a vasculopathy involving multiple organ systems that leads to the presence of cutaneous and mucosal telangiectasias, digital ulcers and tissue ischemia. Medium-size vessels can also be involved, sometimes leading to scleroderma renal crisis or pulmonary artery hypertension. However, scleroderma is rarely associated with an ANCA-positive vasculitis. We report this unique situation.
Case Report
A 53-year-old woman was admitted to our hospital with weakness and numbness in her legs. She had been diagnosed with Raynaud’s phenomenon 10 years before, followed by the development of autoimmune hypothyroidism. Three years later, she complained of telangiectasias spreading over her face and neck, and she developed a digital ulcer on her middle finger. She noted a dry cough and dyspnea on exertion. The ANA and the anti-topoisomerase antibodies (Scl-70) were positive. She was diagnosed with dSS with pulmonary involvement (moderate to severe pulmonary fibrosis).
Launa Cherry says
August 29, 2017 at 11:00 amI went to to the Royal/Barts Neuromusclar Clinic .As I have had trouble with a cough and drinking certain drinks, my anticentromere antibody was positive ANCA;MPO PR3 BEING NEGATIVE. The anticentromere positive antibody is indicative of limited systemic scierosis causing oesophageal dysmotiliity. waiting to see a rheumatologist.
I am 73 in December I walk every where and do tie chi yoga and go to the gym, I used to go for 2 hours but because of What I have I do half to three quaters of the hour,
.I have to take inhalers during the day.I also take Omerprazole one a day Amlodipine
Cetirizine HYDROCHLORIDE one day.Nobody has explained what the above means I have since having a c section in 1975 I had a baby son My blood group changed to O Rhd negative antibodies Anti-k I must have had antibodies then. Please can you send any leaflets on the above.
Keri Losavio says
September 5, 2017 at 12:41 pmPatient information is available on the ACR website. Click here.