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Introduction: Antibrush border antibody disease (ABBA) is an autoimmune tubulointerstitial kidney disease that primarily affects older individuals and results in progressive kidney failure. It is rare with only 20 reported cases. Here, we describe a case series to further define the clinicopathologic spectrum and natural history, and to inform management. Methods: We identified 67 patients with ABBA who underwent kidney biopsy, including 65 native and 2 transplants. Demographics, clinical findings, and laboratory data were obtained. Histopathologic data included light microscopy, immunofluorescence, electron microscopy and immunostaining for LRP2, CUBN, and AMN. Follow-up data, including treatment(s), laboratory values, and outcomes, were available from 51 patients. Results: Patients with ABBA were predominantly male with a median age of 72 years. Median serum creatinine was 2.7 mg/dl, proteinuria was 2.8 g/day, and hematuria was present in two-thirds of the patients. Tubular injury with LRP2-positive tubular basement membrane (TBM) deposits were seen in 94.2% of patients. Thirty-eight patients (56.7%) had a second kidney disease, commonly glomerular diseases with high-grade proteinuria. These diseases included podocytopathies, membranous nephropathy (MN), IgA nephropathy, diabetic glomerulopathy, lupus nephritis (LN), crescentic glomerulonephritis (GN), tubulointerstitial nephritis, and involvement by lymphoma. The majority of patients were treated with immunosuppression. Of those patients with follow-up, 29.4% achieved remission, 70.6% had no response, and 52.8% required dialysis or were deceased. Untreated patients were at the highest risk. Conclusion: ABBA is a rare autoimmune kidney disease that often occurs with other kidney diseases. Although the overall prognosis of ABBA is poor, there is potential benefit from immunosuppression.
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INTRODUCTION: Psoriasis is a chronic inflammatory skin disease characterized by epidermal hyperproliferation, abnormal differentiation and inflammatory infiltration in the dermis. The dermal microvascular expansion associated with abnormal orientation and dilatation of capillaries in the biopsies of the psoriatic skin suggest that the disease is dependent on angiogenesis. AIM: To analyze and compare the immunohistochemical expression of angiogenic factors - Vascular Endothelial Growth Factor (VEGF), von Willebrand Factor (vWF) and CD 34 in skin biopsies of psoriasis cases with control skin samples; and to correlate the expression of angiogenic factors with Psoriasis Area and Severity Clinical Index (PASI SCORE). MATERIALS AND METHODS: This was a prospective case control study conducted over a period of 15 months. Thirty-two psoriasis cases and thirty control skin samples were included in the study. Skin biopsy specimen was taken from clinically diagnosed psoriasis cases who did not receive any treatment. The diagnosis of psoriasis vulgaris was confirmed after microscopic examination. Immunohistochemical expression for VEGF, vWF and CD 34 was studied. RESULTS: VEGF expression in epidermis was significantly higher in cases when compared to control skin (p <0.01). CD 34 expression was significantly upregulated in cases when compared to controls (p<0.01). Von Willebrand factor expression was weak in both the cases and the controls. Significant correlation between the expression of VEGF and PASI score (r=0.944; p<0.01), and expression of CD 34 and PASI score was observed (r=0.942; p<0.01). CONCLUSION: In the present study, significant overexpression of VEGF and CD 34 was noted in cases when compared to controls. The keratinocytes in the psoriatic skin lesions were recognized as a source of pro-angiogenic cytokines namely the VEGF and other growth factors which promotes angiogenesis in psoriatic plaque. Angiogenesis plays an important role in genesis and development of psoriasis vulgaris. Therefore, development of targeted anti-angiogenic therapy might be beneficial for this chronic disabling dermatological disease.