Background: Anti-neutrophil cytoplasm antibodies in necrotizing vasculitides need to be distinguished from ANCAs1 in other inflammatory conditions to avoid clinical misinterpretation.
Objectives: To help clinicians and laboratory scientists recognize and utilize vasculitis-related ANCAs as an aid in diagnostic workup and patient follow-up, and be aware that ANCAs with different characteristics are commonly found in other chronic inflammatory conditions that persistently engage neutrophils in the inflammatory process.
Methods: Indirect immunofluorescence and enzyme immunoassay methods were used to detect ANCAs with both known and unknown neutrophil autoantigenic targets.
Results: Primary necrotizing small vessel vasculitides such as Wegener’s granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and renal-limited rapidly progressive necrotizing glomerulonephritis target either the serine protease proteinase 3 or myeloperoxidase in azurophilic granules. In ulcerative colitis and rheumatoid arthritis, we found multiple ANCA targets contained in azurophilic and specific granules, the cytosol and the nucleus, whereas PR32 and MPO3 were not, or only weakly, recognized.
Conclusions: ANCAs typically found in active SVV4 are demonstrable both by indirect immunofluorescence and antigen-specific enzyme immunoassay, and strong reactivity to either PR3 or MPO is characteristic. Strong ANCA with MPO reactivity is also found in some patients with drug-induced syndromes (lupus, vasculitis). Intermediate to strong perinuclear ANCAs are found in a substantial proportion of patients with UC5 (40–60%) and RA6 (30–70%), but in these conditions the ANCAs have many antigen targets that are only weakly recognized.
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1 ANCA = anti-neutrophil cytoplasm antibody
2 PR3 = protease proteinase 3
3 MPO = myeloperoxidase
4 SVV = small vessel vasculitides
5 UC = ulcerative colitis
6 RA = rheumatoid arthritis
Roberto Spiegelmann, MD, Jana Gofman, MSc, Dror Alezra, MSc and Raphael Pfeffer, MD
Background: Radiosurgery is a therapeutic technique characterized by the delivery of a single high dose of ionizing radiation from an external source to a precisely defined intracranial target. The application of radiosurgery to the treatment of acoustic neurinomas has increased substantially in the last decade. Most of the published experience pertains to the use of the gamma knife.
Objectives: To report the experience at the first Israeli Linear Accelerator Radiosurgery Unit in the management of 44 patients with acoustic neurinomas.
Methods: We analyzed the clinical records and imaging studies of all patients undergoing radiosurgery for acoustic neurinomas between 1993 and 1997, and quanitified the changes in tumor volume, hearing status, and facial and trigeminal nerve function. The contribution of radiation dose and original tumor volume upon those variables was also studied.
Results: At a mean follow-up of 32 months (range 12–60), 98% of the tumors were controlled (75% had shrunk; 23% had stable volume). The actuarial hearing preservation rate was 71%. New transient facial neuropathy developed in 24% of the patients, persisting in mild degrees in 8%. Neuropathy correlated primarily with tumor volume. Tumors with volumes 4 ml were at high risk when marginal radiation doses were 1,400 cGy. Dose reduction to a maximum of 1,400 cGy produced no neuropathies in the last 20 patients, still preserving tumor control rates.
Conclusions: Radiosurgery is an effective and cost-efficient therapeutic modality for newly diagnosed acoustic neurinomas in the elderly or medically infirm population, and for all residual or recurrent tumors after conventional surgery.
Derek Le-Roith, MD, Michael Karas, MD, Shoshana Yakar, MD, Bao-He Qu, MD, Yiping Wu, MD, and Vicky A. Blakesley, MD.
Pnina Langevitz, MD, Avi Livneh, MD, Shai Padeh, MD, Nurit Zaks, MD, Yael Shinar, MD, Deborah Zemer, MD, Elon Pras, MD, and Mordechai Pras, MD.
Sandra Reynoso-Paz, MD, Ross L. Coppel, MD, Aftab A. Ansari MD, and M.Eric Gershwin, MD
Hertzel Salman, MD, Pearl I. Herskovitz, MD, Simcha Brandis, MD, Michael Bergman, MD, Dror Dicker, MD, and Izhar Zahavi, MD.
Joel Sayfan, MD, and Ada Rosen, MD.
Dan Regev, MD, Yoram Wolf, MD, and Daniel Hauben, MD.
Ittai Shavit, MD, Naim Shehadeh, MD, Osnat Zmora, MD, Israela Avidor, MD, and Amos Etzioni, MD.