Browsing by Subject "granulomatosis with polyangiitis"

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    Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis
    (Springer, 2017-03) Kouri, Anne M.; Andreoli, Sharon P.; Department of Pediatrics, IU School of Medicine
    Background Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a small- and medium-sized vasculitis classically seen in adult patients, with peak onset near the fifth to seventh decade of life. There is little data on ANCA-associated vasculitis in pediatric patients, and most studies have limited follow-up. Methods This is a retrospective chart review of 22 patients with ANCA-positive glomerulonephritis in a single institution from 1991 to 2013. Results Of the 22 patients, eight (36 %) required renal replacement therapy (RRT) at diagnosis; four of these patients recovered sufficient renal function to initially discontinue dialysis. Five patients (23 %) were treated with plasmapheresis at presentation. The median time from presentation until first clinical or serologic relapse was 1.7 ± 1.2 years. After a median follow-up of 5.8 years, just over half of our patients had chronic kidney disease (CKD) stages 1–3 (55 %). Seven (32 %) patients progressed to end-stage renal disease (ESRD) and eventually required kidney transplant. Conclusion ANCA-associated glomerulonephritis is a rare disorder in children. Presentation and outcomes vary significantly among patients. More research is required to follow these patients who are diagnosed in childhood to further characterize the long-term outcome of the disease.
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    Macula Retinal Vasculitis and Choroiditis Associated with Granulomatosis with Polyangiitis
    (Wolters Kluwer, 2017) Lim, Lik Thai; Vasudevan, Vinaya; Shelton, Julie; Vitale, Albert T.; Moorthy, Ramana; Ophthalmology, School of Medicine
    Purpose: To report a case series of two cases of granulomatosis with polyangiitis, previously known as Wegener granulomatosis, which developed macular necrosis, not previously associated with granulomatosis with polyangiitis, healed with fibrosis, despite aggressive immune-modulating therapy and good control of systemic disease. Methods: Case series of two cases with observation of treatment progress. Results: The results reported the progress of response to treatment in the two cases, which resulted in the final outcome of fibrosis in the macula region, despite being on aggressive immune-modulating therapy and good systemic control. Conclusion: Granulomatosis with polyangiitis can be associated with macular necrosis leading to fibrosis, unresponsive to immune-modulating therapy.
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    Vasculitis manifested with multiple mass lesions in kidneys, lungs and soft tissue, mimicking malignant tumors
    (Elsevier, 2019-09) Guo, Shunhua; Domingo, Mary Ann R.; Chang, Qin; Swensson, Jordan K.; Pathology and Laboratory Medicine, School of Medicine
    We report a case of granulomatosis with polyangiitis (GPA) (Wegner's granulomatosis) who presented with multiple mass lesions in kidneys and lung lobes, as well as neck soft tissue, mimicking malignancies. This 71-year-old woman initially presented with sudden right foot drop, left calf pain and right eye vision loss. She was treated with corticosteroid for the diagnosis of possible temporal arteritis. Months after steroid was tapered to 2 mg per day, she developed increasing fatigue, weight loss, and shortness of breath. CT scan showed lung mass lesions in left upper lobe (3.8 × 2.4 cm), right mid lung with pleural extension (3.4 × 3.3 cm), and right lower lobe (1.1 × 1.0 cm); right neck (3.3 × 2.6 cm), right kidney (2.3 × 1.8 cm) and left kidney (2.0 × 1.6 cm). Right quadriceps muscle biopsy shows focal granulomatous inflammation. Lung biopsy showed necrotizing and poorly formed granulomatous inflammation. Biopsies of kidney mass lesions showed necrotizing and non-necrotizing granulomatous inflammation. No crescentic or necrotizing glomerular lesions were observed in the total 40 sampled glomeruli. No malignancy was identified in any of the biopsies. Her c-ANCA was found to be positive and PR3-ANCA antibody was 6.88 U/ml (normal 0–0.90 U/ml). She was diagnosed with granulomatosis with polyangiitis and treated with high dose corticosteroid and rituximab. Eight months later, follow-up showed resolved mass lesions by chest X-ray and CT and stable renal function. The case highlights the atypical clinical presentation of vasculitis and the significance of considering this possibility in differential diagnosis when confronting mass lesions present in multiple organ systems. Biopsy is critical for the correct diagnosis to initiate timely and appropriate treatment, and also important to avoid unnecessary surgical resection.