Scleroderma-associated thrombotic microangiopathy in overlap syndrome of systemic sclerosis and systemic lupus erythematosus

Abstract

Rationale: Systemic sclerosis (SSc) is a serious multisystem connective tissue disease. When SSc is accompanied by systemic lupus erythematosus (SLE), called SSc-SLE overlap syndrome. SSc associated thrombotic microangiopathy (SSc-TMA) can lead to scleroderma renal crisis, it mainly manifests hypertension or even malignant hypertension, acute kidney injury, and higher mortality. The case of SSc-SLE overlap syndrome combined with SSc-TMA has rarely been reported. Patient concerns: We report the case of an elderly male with SSc-SLE overlap syndrome combined with scleroderma renal crisis and SSc-TMA. Diagnoses: The patient has typical of SSc on the face and hands, combined with pulmonary artery hypertension, interstitial lung disease, heart failure and malignant hypertension, as well as SLE, lupus nephritis class V, and TMA, which were definitively diagnosed by clinical laboratory examination and renal histopathology. Interventions: The patient was treated with prednisone, cyclophosphamid, renin-angiotensin system inhibitors, diuretics, and acetylcysteine. Outcomes: The patient died suddenly of heart failure on the 35th day after discharge. Lessons: The occurrence of TMA leads to the deterioration of the prognosis of SSC-SLE overlap syndrome. The diagnosis of SSC-TMA in SSc-SLE overlap syndrome depends on clinical laboratory examination and renal histopathology.

Systemic sclerosis complicated with renal thrombotic microangiopathy: a case report and literature review

“…They showed different prognosis. Two reported cases (case 3 [ 24 ] and case 5 [ 25 ]) became HD dependent during follow up, two cases (case 1 [ 26 ] and case 4 [ 27 ]) died because of severe complications of diffused alveolar hemorrhage and heart failure, and one case (case 2 [ 28 ]) showed recovery to normal kidney function. The case with good prognosis presented acute tubular necrosis and mild TMA change in renal biopsy.…”

Systemic Lupus Erythematosus in Men: Brief Description of Clinical and Laboratory Findings

“…Predominantly occurs in young and middle-aged women. Approximately 6.8% to 14.7% of SSc patients have overlapping systemic lupus erythematosus (SLE), called SSc-SLE overlap syndrome [32].…”