In Clinical Case Reports, physicians presented the case report of a patient with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who developed corneal melt and perforation. 

A 75-year-old man presented with palpitations and dyspnea. He had a history of multiple surgeries for appendicitis, hemorrhoids, rectal cancer, and inguinal herniation. He was a smoker from 20 to 40 years of age and was on medication for atrial fibrillation diagnosed 2 years ago. 

Upon admission, the patient developed fever and had elevated C-reactive protein levels that did not resolve with oral or intravenous antibiotics. Blood and urine cultures were negative. He was referred to the University Hospital of Kawasaki Medical School. 

Upon referral, the patient demonstrated jaw claudication, as well as cervical and pharyngeal pain. He also complained of hip joint pain and pain in his lower extremities. He had lost 3 kg in a period of 2 weeks. Laboratory investigations raised clinical suspicions of temporal arteritis, which prompted his physicians to perform a biopsy of the left temporal artery, revealing small branch vessel fibrinoid necrosis and perivascular infiltration of mononuclear cells. 

Read more about AAV etiology 

In view of these findings and the patient’s myeloperoxidase (MPO)-ANCA levels of 19.8 U/mL, he was diagnosed with microscopic polyangiitis and was started on oral prednisolone. His MPO-ANCA levels rose to 129 U/mL in 6 months. 

One year after his initial presentation, the patient was started on azathioprine and sulfamethoxazole/trimethoprim, but these were discontinued due to adverse reactions. He was then prescribed azathioprine only with prednisolone, which resulted in a decrease in serum MPO-ANCA levels to less than 10 U/mL. 

Five and a half years after his initial visit, the patient developed bulbar conjunctival infection and corneal haze bilaterally. An eye examination revealed diffuse scleritis in both eyes with peripheral corneal infiltration. In view of these ocular manifestations of AAV, the patient was referred to rheumatology. 

Approximately 1 month later, the patient’s left eye showed impending corneal melt, while his right eye showed central corneal perforation with iris incarceration and active scleritis. He was started on steroid mini-pulse therapy together with azathioprine, which stabilized his condition. He was later prescribed rituximab and underwent cataract surgery with intraocular lens implantation. 

The patient died from pneumonia shortly after. 

The authors of the report emphasized the importance of continued ophthalmic surveillance among patients with AAV with the goal of preventing ocular deterioration. 


Matsuo T, Hiramatsu-Asano S, Sawachika H, Nishimura H. ANCA-associated vasculitis with scleritis, corneal melt, and perforation rescued by rituximab: case report and literature reviewClin Case Rep. Published online June 20, 2023. doi:10.1002/ccr3.7595