Rare Inflammatory Disorder of Brain Meninges Linked to MPO-positive AAV

A rare case of inflammatory disease affecting the protective membrane of the central nervous system was linked to anti-myeloperoxidase (MPO) autoantibodies in a case report. The Japanese study, “A case of suspected MPO-ANCA-related hypertrophic pachymeningitis with atypical presentation,” was published in the Journal of Family and…

This week marks the launch of the “7,000 Mile Rare Movement,” a nationwide effort to raise money for research into the 7,000 known rare diseases that afflict at least 30 million Americans. The campaign kicks off Feb. 1 and culminates with Rare Disease Day on Feb. 28. Organized by…

A new study found why some patients with ANCA-associated vasculitis (AAV) have autoantibodies in the blood, but not at the sites of injury. This matter has puzzled scientists for a long time. While MPO autoantibodies are needed to induce vasculitis in such patients, the cells causing the injury produce an…

Prolonged exposure to the silica dust that construction and other industries generate contributes to the development of the small blood vessel disease known as microscopic polyangiitis, according to a Japanese case study. Microscopic polyangiitis, or MPA, is one of three types of a category of diseases known as ANCA-associated vasculitis, or…

A man who had granulomatosis with polyangiitis and small lung lesions bled in the air sacs of his lungs after doctors treated him for his inflammatory blood vessel condition, a Japanese case study shows. Essentially, this meant that his condition had taken a turn for the worse. A mix of…

A case report of a patient with granulomatosis with polyangiitis (GPA) along with ocular involvement suggests that Rituxan (rituximab) might be successful for people who don’t respond to prednisolone and cyclophosphamide. The study, “Peripheral Ulcerative Keratitis Associated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab,”…

Rituxan (rituximab) treatment is effective in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who failed to respond to standard immunosuppressors, results from a Turkish study suggest. But the team at Istanbul University noted that key data on its use — especially long-term data — is still lacking. The…

Researchers have identified a fat molecule that contributes to kidney damage in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, or AAV. The molecule, sphingosine 1-phosphate (S1P), works to enhance the damaging effects of MPO autoantibodies, one of the two most common disease-causing antibodies produced by AAV patients. The study, “…

ANCA vasculitis may trigger inflammation in large blood vessels as well as the small ones the disease is associated with, Japanese researchers reported after noting that three patients had developed an aortic aneurysm. These patients had ANCA vasculitis caused by myeloperoxidase (MPO) antibodies. An aneurysm is an enlarged segment of the…

Patients with severe kidney injury stemming from anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may benefit from intravenous glucocorticoid pulses, a study indicates. The high-dose treatment helped Chinese patients get off dialysis and showed a tendency toward  increasing their survival, researchers found. They published their findings in the journal BMC Nephrology.