IgG4-rich reactive lymphoid hyperplasia of the lacrimal gland.

We describe an otherwise healthy 24-year-old woman with reactive lymphoid hyperplasia of one lacrimal gland. She was found to have modest elevation of serum IgG4 and high density of IgG4+ plasma cells in her lacrimal gland, ranging from 40% to 60% of IgG+ plasma cells. The patient transiently responded to systemic corticosteroids before treatment with rituximab. IgG4-rich reactive lymphoid hyperplasia of the lacrimal gland is neither an established part of IgG4-related disease spectrum nor a known precursor lesion to lymphoma. Although there is a plausible risk of transforming to IgG4-related disease or to lymphoma, the biological potential and natural history of IgG4-rich reactive lymphoid hyperplasia remains to be determined.

IgG4-related lacrimal sac diverticulitis.

IgG4-related disease is characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. We herein report a case of lacrimal sac diverticulitis with marked IgG4-positive plasma cell infiltration. An 89-year-old woman presenting with right lower eyelid mass. Imaging modalities demonstrated a cystic orbital mass located beneath the globe and adjacent to enlarged lacrimal sac. Serological tests showed high IgG4 and normal IgG levels, measuring 242 and 1603 mg/dl, respectively. The orbital mass was surgically excised. Histologically, the excised tissue demonstrated marked inflammation with fibrosis surrounded by mononuclear epithelial cells. A variety of IgG and IgG4-positive plasma cells infiltrated the stroma. This patient was diagnosed as an IgG4-related lacrimal sac diverticulitis, based on current diagnostic criteria of IgG4-related disease. It is likely that IgG4-related inflammation occurs in a lacrimal sac diverticulum, which should be considered a differential diagnosis in inferior orbital tumors.

Ocular Manifestations of COVID-19 (SARS-CoV-2): A Critical Review of Current Literature.

The novel coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), emerged in China in the city of Wuhan in December of 2019 and since then more than 5,000,000 people have been infected, with approximately 338,000 deaths worldwide. The virus causes the coronavirus disease 2019 (COVID-19), which is characterized by fever, myalgia and cough, with severe acute respiratory syndrome being the most fearsome complication. Nevertheless, the vast majority of cases present mild symptoms or none. Central nervous system and cardiovascular manifestations have been reported. The range of ocular manifestations, either as a result of the infection or as a result of the treatment, has not yet been discussed. In this study, a systematic review of current literature relevant to COVID-19 was performed with focus on modes of transmission, ocular manifestations related to infection and medications, as well as the control of infection in ophthalmic practice.

Clinicoserological factors associated with response to steroid treatment and recurrence in patients with IgG4-related ophthalmic disease.

PURPOSE: To investigate the factors associated with response to steroid treatment and recurrence in patients with IgG4-related ophthalmic disease (ROD). METHODS: Twenty-eight patients with biopsy-proven IgG4-ROD treated between March 2010 and January 2017 were included in this retrospective study. Clinical features, serum IgG4 levels, systemic involvement, treatments and treatment outcome, factors associated with response to treatment and recurrence were assessed. RESULT: Thirteen men and 15 women (mean age 50.8 years) were evaluated over mean follow-up period of 27.3 months. Elevated serum IgG4 levels (>1.35 g/L) and systemic disease were noted in 9 (32%) and 18 patients (64%), respectively. The lacrimal gland was involved in all patients, and 22 patients (78.6%) had bilateral involvement. Most patients (82%) responded well to systemic steroids, but 12 (43%) relapsed after the initial steroid treatment, requiring additional therapies to achieve remission. Complete response to initial steroid treatment was associated with elevated serum IgG4 levels before treatment (P=0.001) and bilateral orbital involvement (P=0.050). Recurrence was associated with elevated serum IgG4 levels before treatment (P=0.007), lower dose (P=0.057) and shorter duration of initial steroids (P=0.042). Patients with recurrence eventually required significantly more steroids than those without recurrence (P=0.011). CONCLUSIONS: Patients with IgG4-ROD responded well to systemic steroid treatment, but recurrence was common, particularly among those with elevated serum IgG4 levels and shorter duration of initial steroid treatment. Low-dose maintenance treatment with systemic steroids should be considered to avoid recurrence in patients with elevated serum IgG4 levels.

Endonasal laser dacryocystorhinostomy: its role in anticoagulated patients.

OBJECTIVE: To report the results of endoscopic laser-assisted dacryocystorhinostomy in anticoagulated patients. STUDY DESIGN: A retrospective study of 16 consecutive anticoagulated patients with distal nasolacrimal duct obstruction treated by endoscopic laser-assisted dacryocystorhinostomy. METHODS: A case note review was made of all patients treated with endoscopic laser-assisted dacryocystorhinostomy who were taking coumadin in two centers between 1993 and 2000. The parameters of age, gender, indications for surgery, surgical findings, complications, and outcome were analyzed. The mean follow-up time was 14 months (range, 9-26 mo). RESULTS: Fifteen of the 16 patients who were treated had an eventual successful outcome, but 6 patients required revision surgery. The patient whose symptoms were not improved was shown to have functional epiphora. No patient had a problem with primary or secondary epistaxis, and no patient required admission. A major benefit was the lack of disruption of anticoagulant therapy. CONCLUSIONS: Endoscopic laser-assisted dacryocystorhinostomy is a safe, efficient technique for the relief of distal nasolacrimal duct obstruction in anticoagulated patients. Not only does it avoid any disruption to their anticoagulant therapy, but it also can be performed as an outpatient procedure.

The autoimmune nature of aqueous tear deficiency.

Twenty-two patients with aqueous tear deficiency (ATD) were examined for the presence of the following autoantibodies: immunofluorescent antinuclear antibody (ANA) and Sjögren's syndrome antibodies A and B (SS-A and SS-B). These autoantibodies were found in 17 (82%) patients but not in control subjects, and they correlated with the severity of symptoms and ocular surface changes. Bacterial keratitis, often recurrent and bilateral, and progressive sterile corneal stromal melting developed in six autoantibody-positive ATD patients. Eight antibody-positive patients had labial salivary or lacrimal gland biopsies, and all showed similar histologic features with marked destruction of the glandular architecture by lymphocytic infiltration. Immunoglobulin and complement were not detected in the glandular tissue. Circumstantial evidence suggests that an abnormal immunologic reaction, possibly related to Epstein-Barr viral (EBV) infection, is the cause of the glandular destruction and tear deficiency.