ABSTRACT
A 47-year-old woman presented with facial spasm, swollen fingers and Raynaud's phenomenon due to cerebrovascular disorder and mixed connective tissue disease (MCTD). Although she was positive for both antineutrophil cytoplasmic antibodies against proteinase-3 (PR3-ANCA) and anti-U1 RNP antibodies, she did not meet the American College of Rheumatology classification criteria for Wegener's granulomatosis (WG). Physical and histopathological examinations revealed severe systemic atherosclerosis without any of the traditional risk factors. Elevated levels of malondialdehyde-modified LDL and antioxidized LDL autoantibodies, which are considered to be key factors in the pathogenesis of atherosclerosis, were also detected in the serum of this patient. In this case, systemic atherosclerosis might have been linked to these autoimmune reactions.
Subject(s)
Antibodies, Antineutrophil Cytoplasmic/immunology , Arteriosclerosis/complications , Mixed Connective Tissue Disease/immunology , Serine Endopeptidases/immunology , Arteriosclerosis/pathology , Aspirin/therapeutic use , Autoantibodies/immunology , Cholesterol, LDL/blood , Female , Granulomatosis with Polyangiitis/immunology , Humans , Lipid Peroxidation , Malondialdehyde/metabolism , Middle Aged , Mixed Connective Tissue Disease/drug therapy , Mixed Connective Tissue Disease/pathology , Myeloblastin , Prednisolone/therapeutic use , Probucol/therapeutic use , Serine Endopeptidases/blood , Treatment Outcome , Valproic Acid/therapeutic useABSTRACT
We report the case of a 49-year-old man who presented with pneumatosis cystoides intestinalis associated with polymyositis and interstitial pneumonia. Three months after the administration of prednisolone and cyclosporine, he noticed mild abdominal distension, and a radiographic examination showed intraperitoneal free gas and intramural gas, suggestive of pneumatosis cystoides intestinalis (PCI). Additional treatment with a combination of doxycycline and high-dose oxygen therapy was effective in relieving the clinical symptoms and reducing the intramural gas.