Key Clinical Message: Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, specifically with myeloperoxidase (MPO)-ANCA, would have a risk for developing corneal melt and perforation abruptly in a short period. It is desirable to have a team of collaboration of rheumatologists and other specialties.
Abstract: An 80-year old man who had been diagnosed 5.5 years previously as ANCA-associated vasculitis by temporal artery biopsy developed corneal melt and perforation with scleritis in both eyes. He underwent successful cataract surgery and retained ambulatory vision with the aid of intravenous rituximab. Two additional patients with similar manifestations were found in the literature.
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http://dx.doi.org/10.1002/ccr3.7595 | DOI Listing |
BMJ Case Rep
January 2025
Dermatology, The Royal Melbourne Hospital, Parkville, Victoria, Australia.
Granulomatosis with polyangiitis (GPA) is a small-to-medium vessel vasculitis that can affect the skin, respiratory tract, kidneys and other organs. A rare cutaneous manifestation of GPA is pyoderma gangrenosum (PG)-like ulcerations, which can have debilitating and disfiguring consequences. We report the case of a man in his 40s with refractory PG-like ulcerations secondary to GPA, not responsive to conventional immunosuppression, who was successfully treated with rituximab.
View Article and Find Full Text PDFCureus
December 2024
Department of Orthopaedics, Tokyo Metropolitan Bokutoh Hospital, Tokyo, JPN.
Hypertrophic pachymeningitis (HP) is a rare inflammatory disease that causes the thickening of the dura mater. Its etiology is mainly classified as idiopathic or secondary, and autoimmune disease is one of the main causes of secondary HP. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-related disease are common among autoimmune diseases.
View Article and Find Full Text PDFDrug Des Devel Ther
January 2025
Center of Expertise for Lupus-, Vasculitis- and Complement-Mediated Systemic Diseases (Luvacs), Department of Internal Medicine - Nephrology Section, Leiden University Medical Center, Leiden, the Netherlands.
Recently, avacopan has been approved for the treatment of ANCA-associated vasculitis (AAV). Avacopan is an inhibitor of the C5a-receptor, which plays an important role in chemotaxis and the amplification loop of inflammation in AAV. In the most recent, international guidelines avacopan is recommended as steroid-sparing agents for the management of AAV.
View Article and Find Full Text PDFKorean J Neurotrauma
December 2024
Department of Neurosurgery, Kyungpook National University Hospital, Kyungpook National University School of Medicine, Daegu, Korea.
This case report describes a rare presentation of antineutrophil cytoplasmic antibodies-associated hypertrophic pachymeningitis in a 49-year-old woman with cranial and spinal lesions. Initial management comprised decompressive laminectomy and steroid therapy, with the subsequent addition of immunosuppressant therapy following symptom recurrence. After a ten-year follow-up, significant symptom improvement and return of motor function were noted.
View Article and Find Full Text PDFTypical renal involvement of antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is pauci-immune glomerulonephritis that presents clinically as rapidly progressive renal failure (RPRF). Here, we report an unusual presentation of myeloperoxidase (MPO)-specific ANCA with isolated involvement of the tubulointerstitium in the form of peritubular capillaritis as the sole lesion without any involvement of the glomerulus. A 52-year-old woman with no previous comorbidities presented with nonspecific symptoms such as fatigue, dysuria, and nausea for two months.
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