Intro: Primary Sjogren’s syndrome is a chronic systemic autoimmune disease. Although ocular and oral dryness are the hallmarks of this syndrome, renal involvement when present most commonly has the histological appea...Intro: Primary Sjogren’s syndrome is a chronic systemic autoimmune disease. Although ocular and oral dryness are the hallmarks of this syndrome, renal involvement when present most commonly has the histological appearance of tubointerstitial nephritis and less often glomerular involvement. We report, a unique case of renal manifestations of primary Sjogren’s syndrome with a proven histological focal proliferative and crescentric necrotizing glomeru-lonephritis. Case: A 30 years old man with Kimura disease presented with four months of subjective fevers, migrating skin erythema, dry mouth, and dry eyes. On further workup the patient was found to have a positive ANA, positive anti-SSA, and was evaluated by ophthalmology who found keratoconjunctivitis sicca. The patient was started on hydroxychloroquine in combination with prednisone. On follow up, the patient was found to be hypertensive with acute kidney injury, a creatinine of 3.65, proteinuria and hematuria. Renal ultrasound was unremarkable. Cytoplasmic ANCA was positive and kidney biopsy was performed revealing non pauci-immune focal proliferative and necrotizing glomerulonephritis with cellular crescents and mesangial dense deposits by electron microscopy. Conclusion: To our knowledge, the association between biopsy proven focal proliferative and necrotizing glomerulonephritis with primary Sjogren’s syndrome has never been previously described in the literature, nor has there been a reported case of association between Kimura disease, ANCA serology and primary Sjogren’s syndrome.展开更多
Red eye is common in our daily practice.It ranges from non-inflammatory to inflammatory causes.An extended course of disease should prompt suspicion and the possibility of diagnosis revision.A prolonged conjunctivitis...Red eye is common in our daily practice.It ranges from non-inflammatory to inflammatory causes.An extended course of disease should prompt suspicion and the possibility of diagnosis revision.A prolonged conjunctivitis mimicking nodular episcleritis can be presented as a manifestation of granulomatosis with polyangiitis(GPA).A 57-year-old woman complained of eye redness and tearing for two weeks which partially resolved with antibiotics.She was subsequently commenced on topical and oral non-steroidal anti-inflammatory drugs(NSAIDs)and topical anti-allergic.However,in the following reviews she developed cornea thinning and her systemic examination revealed an injected uvula with absence of upper respiratory tract infection.She was investigated for connective tissue disease and found to have raised anti-inflammatory markers and her antinuclear antibody and C-ANCA tests were positive.She was diagnosed with GPA.Her conditions improved followed by the commencement of topical corticosteroid with high dose of systemic corticosteroid,which followed by a tapering regime with oral corticosteroid.Although red eye is common,it is associated with a variety of diseases.GPA manifestation can be as subtle as a red eye.Any prolonged partially treated red eye should prompt suspicion of a more sinister cause.Sensitive detection of other subtle systemic signs is very important.展开更多
文摘Intro: Primary Sjogren’s syndrome is a chronic systemic autoimmune disease. Although ocular and oral dryness are the hallmarks of this syndrome, renal involvement when present most commonly has the histological appearance of tubointerstitial nephritis and less often glomerular involvement. We report, a unique case of renal manifestations of primary Sjogren’s syndrome with a proven histological focal proliferative and crescentric necrotizing glomeru-lonephritis. Case: A 30 years old man with Kimura disease presented with four months of subjective fevers, migrating skin erythema, dry mouth, and dry eyes. On further workup the patient was found to have a positive ANA, positive anti-SSA, and was evaluated by ophthalmology who found keratoconjunctivitis sicca. The patient was started on hydroxychloroquine in combination with prednisone. On follow up, the patient was found to be hypertensive with acute kidney injury, a creatinine of 3.65, proteinuria and hematuria. Renal ultrasound was unremarkable. Cytoplasmic ANCA was positive and kidney biopsy was performed revealing non pauci-immune focal proliferative and necrotizing glomerulonephritis with cellular crescents and mesangial dense deposits by electron microscopy. Conclusion: To our knowledge, the association between biopsy proven focal proliferative and necrotizing glomerulonephritis with primary Sjogren’s syndrome has never been previously described in the literature, nor has there been a reported case of association between Kimura disease, ANCA serology and primary Sjogren’s syndrome.
文摘Red eye is common in our daily practice.It ranges from non-inflammatory to inflammatory causes.An extended course of disease should prompt suspicion and the possibility of diagnosis revision.A prolonged conjunctivitis mimicking nodular episcleritis can be presented as a manifestation of granulomatosis with polyangiitis(GPA).A 57-year-old woman complained of eye redness and tearing for two weeks which partially resolved with antibiotics.She was subsequently commenced on topical and oral non-steroidal anti-inflammatory drugs(NSAIDs)and topical anti-allergic.However,in the following reviews she developed cornea thinning and her systemic examination revealed an injected uvula with absence of upper respiratory tract infection.She was investigated for connective tissue disease and found to have raised anti-inflammatory markers and her antinuclear antibody and C-ANCA tests were positive.She was diagnosed with GPA.Her conditions improved followed by the commencement of topical corticosteroid with high dose of systemic corticosteroid,which followed by a tapering regime with oral corticosteroid.Although red eye is common,it is associated with a variety of diseases.GPA manifestation can be as subtle as a red eye.Any prolonged partially treated red eye should prompt suspicion of a more sinister cause.Sensitive detection of other subtle systemic signs is very important.
