Τίτλος:
A case report of hypocomplementemic urticarial vasculitis presenting with membranoproliferative glomerulonephritis
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Background: Hypocomplementemic urticarial vasculitis syndrome is an infrequent condition characterized by ocular, renal, gastrointestinal and pulmonary involvement with low serum complement levels and autoantibodies. Renal manifestations vary from microscopic hematuria to nephrotic syndrome and acute kidney injury. Accordingly differing histologic patterns have been reported. Case presentation: We present the case of a 65 years old woman with a history of chronic uveitis who presented with arthralgias, urticarial rush, nephrotic syndrome, glomerular hematuria and low serum complement. Kidney biopsy revealed an immune-complex membranoproliferative glomerulonephritis. The patient received induction therapy with steroids, cyclophosphamide and hydroxychloroquine followed by rapid clinical improvement and remission of proteinuria. Maintenance treatment consisted of rituximab pulses. Conclusions: The majority of hypocomplementemic urticarial vasculitis syndrome cases is idiopathic, although an association to drugs, infections or other autoimmune disorders has been recorded. Given the rarity and heterogeneity of the disease, no standard treatment is established. © 2020 The Author(s).
Συγγραφείς:
Vallianou, K.
Skalioti, C.
Liapis, G.
Boletis, J.N.
Marinaki, S.
Περιοδικό:
BMC Nephrology
Εκδότης:
BioMed Central Ltd.
Λέξεις-κλειδιά:
albumin; amlodipine; antihistaminic agent; C reactive protein; complement component C1q; complement component C3; complement component C4a; creatinine; cryoglobulin; cyclophosphamide; hemoglobin; hemoglobin A1c; hydroxychloroquine; immunoglobulin G; immunoglobulin M; levothyroxine; metformin; methylprednisolone; nebivolol; omalizumab; rituximab; steroid; valsartan; antirheumatic agent; complement; glucocorticoid, aged; arthralgia; arthritis; Article; case report; chronic obstructive lung disease; chronic urticaria; clinical article; creatinine blood level; diabetes mellitus; drug withdrawal; erythrocyte; erythrocyte sedimentation rate; eye examination; female; forced expiratory volume; forced vital capacity; Hashimoto disease; hematuria; histopathology; human; human cell; human tissue; hypertension; hypoalbuminemia; hypocomplementemia; hypocomplementemic urticarial vasculitis syndrome; immunofluorescence; kidney biopsy; leg edema; leukocytoclastic vasculitis; lung function test; membranoproliferative glomerulonephritis; microscopy; nephrotic syndrome; physical examination; proteinuria; pruritus; rash; skin biopsy; urticaria; uveitis; arthritis; chronic urticaria; complication; membranoproliferative glomerulonephritis; metabolism; nephrotic syndrome; pathology; proteinuria; uveitis; vasculitis, Aged; Antirheumatic Agents; Arthritis; Chronic Urticaria; Complement System Proteins; Cyclophosphamide; Female; Glomerulonephritis, Membranoproliferative; Glucocorticoids; Humans; Hydroxychloroquine; Nephrotic Syndrome; Proteinuria; Rituximab; Uveitis; Vasculitis
DOI:
10.1186/s12882-020-02001-6
