1/4. Orbital inflammatory pseudotumor and ischemic vasculitis in churg-strauss syndrome: report of two cases and review of the literature.OBJECTIVE: To clarify the characteristics of ocular manifestations in churg-strauss syndrome (allergic granulomatosis and angiitis). DESIGN: Two interventional case reports and literature review. PARTICIPANTS: Two patients with churg-strauss syndrome with ocular manifestations are described; 15 previously reported cases and the present 2 cases of churg-strauss syndrome with ocular manifestations are reviewed. INTERVENTION: Ocular manifestations were divided into two groups: orbital inflammatory pseudotumor and ischemic vasculitis. MAIN OUTCOME MEASURES: The onset, conjunctival involvement, orbital imaging, antineutrophil cytoplasmic antibodies (ANCA), and visual prognosis were evaluated. RESULTS: The characteristics of the orbital inflammatory pseudotumor type (eight cases) are chronic onset, positive conjunctival involvement, abnormalities in orbital imaging studies, negative ANCA, and good visual prognosis. The ischemic type (nine cases) is characterized by sudden onset, no conjunctival involvement or abnormalities in imaging studies, positive ANCA, and occasional poor visual prognosis. CONCLUSIONS: Orbital inflammatory pseudotumor and ischemic vasculitis may represent two essential characteristics of churg-strauss syndrome, granulomatosis and angiitis, respectively. The clinical features of the two types are so distinct that differentiation may be meaningful for diagnosis and treatment of churg-strauss syndrome with ocular manifestations.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
2/4. Vasculitis of the lacrimal sac wall in wegener granulomatosis.A 35-year-old woman with a 4-year history of generalized wegener granulomatosis (WG) had clinically controlled disease. She was evaluated for a 6-month history of right lacrimal sac mass. On examination, a right chronic dacryocystitis and mucocele were observed. A right external dacryocystorhinostomy was performed. The surgical biopsy specimen from the lacrimal sac showed leukocytoclastic vasculitis with more aggressive damage to the small vessels in the deeper mucosa and focal microhemorrhages. The patient was free of symptoms 1 year after surgery. We believe this is the first report of generalized WG presenting features of an active vasculitis of the lacrimal sac wall on surgical biopsy specimen. We conclude that the lacrimal drainage system can be affected directly by focal WG vasculitis, suggesting that nasolacrimal duct obstruction is not always due to contiguous paranasal disease.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
3/4. Recurrent bilateral dacryocystoceles in Wegener's granulomatosis: a rhinologic perspective.Wegener's granulomatosis (WG) is a rare, idiopathic, systemic vasculitis of small vessels that manifests in multiple organ systems. Otorhinolaryngic manifestations of this disease include recurrent sinusitis and relapsing polychondritis. Periocular involvement is also a well-documented location of Wegener's disease. We present the case of a 13-year-old girl with severe WG who developed multiple recurrent orbital infections. She underwent multiple incision and drainage surgeries of each orbit and multiple courses of intravenous antibiotics. The patient persistently reaccumulated purulence in her nasolacrimal duct system and was referred to an oculoplastic surgeon for evaluation of these recurrent infections. The diagnosis of dacryocystitis as a complication of WG was made. This unique case represents a patient with severe WG developing bilateral dacryocystitis requiring bilateral dacryocystorhinostomies.- - - - - - - - - - ranking = 0.16666666666667keywords = vasculitis (Clic here for more details about this article) |
4/4. Wegener's granulomatosis presenting as dacryoadenitis.Wegener's granulomatosis is a life-threatening condition characterized by the triad of necrotizing granulomatous inflammation of the respiratory tract, vasculitis, and glomerulonephritis. During early stages of the disease, some patients may present with only one or two components of the triad. Other patients may be affected by the limited form of Wegener's which lacks the renal involvement. We report an 18-year-old female who initially presented with bilateral dacryoadenitis. A lacrimal gland biopsy was consistent with Wegener's granulomatosis. A few months later she developed glomerulonephritis and responded well to cyclophosphamide treatment. Wegener's granulomatosis remains a diagnostic challenge and is frequently underdiagnosed. early diagnosis is crucial in saving many lives because the disease is potentially curable with cytotoxic therapy. Dacryoadenitis may be one of the initial presentations of Wegener's granulomatosis.- - - - - - - - - - ranking = 0.16666666666667keywords = vasculitis (Clic here for more details about this article) |