Vascular Occlusion in Kidney Biopsy Is Characteristic of Clinically Manifesting Thrombotic Microangiopathy

Abstract

Thrombotic microangiopathy (TMA) can sometimes manifest only histologically. Our aim was to retrospectively compare biopsy-proven adult TMA patients showing only histological (h-TMA) or both histological and clinical (c-TMA) TMA in 2006–2017. All native kidney biopsies with TMA were included. Biopsies were re-evaluated by light and electron microscopy, and immunofluorescence. Clinical characteristics, laboratory variables, and treatments were recorded from the electronic medical database. Patients were categorized into h-TMA and c-TMA and these groups were compared. In total, 30 biopsy-proven cases among 7943 kidney biopsies were identified and, of these, 15 had h-TMA and 15 c-TMA. Mean follow-up was 6.3 y, and 73.3% had secondary hemolytic uremic syndrome (HUS) and the rest were atypical HUS. Patient characteristics, treatments, and kidney, and patient survival in the groups were similar. Statistically significant differences were found in histological variables. Vascular myxoid swelling and vascular onion-skinning were almost exclusively detected in c-TMA and, thus, vascular occlusive changes indicate clinically apparent rather than merely histological TMA. In addition, regardless of clinical presentation, kidney and patient survival times were similar in the patient groups highlighting the importance of a kidney biopsy in the case of any kidney-related symptoms.

Thrombotic microangiopathy in IgA nephropathy: pathophysiology, histopathology, and treatment perspectives

“…Histologically, TMA in IgAN is characterized by fresh fibrinous vascular thrombi, distinguished by the presence of bright reddish staining fibrinous material and marked dilation with smoothing of the internal elastic lamina (45,46). Chronic lesions exhibit organized thrombi with recanalized vascular channels, often displaying an "onion-skin" appearance (47,48). Correspondingly focal myocyte necrosis may be present, often in association with thrombi (2,42).…”

Clinical Evaluation and Management of Thrombotic Microangiopathy

“…This is especially true in subacute or chronic TMA, which may represent a smoldering or previously active process. 6 A thorough history and physical examination are important to identify coexisting conditions, family history, and drug exposures. Clinical features alone, however, cannot reliably differentiate the TMA etiologies.…”

“…Individual features vary, 3,4 and TMA can be identified on tissue biopsy even in the absence of hematologic abnormalities. This is especially true in subacute or chronic TMA, which may represent a smoldering or previously active process 6 …”