Anti-RNA polymerase III antibody-associated scleroderma renal crisis in a patient with limited cutaneous systemic sclerosis: A case report

Abstract

A 69-year-old Japanese man was presented with hypertensive crisis. Renal histology revealed malignant nephrosclerosis, including an onion skin pattern with fibrinoid necrosis of the small arteries from arterioles up to interlobular arteries. Immunological investigation clarified positive anti-RNA polymerase (RNAP) III antibody, and limited cutaneous systemic sclerosis (Lc SSc) was diagnosed by skin biopsy as the underlying disease causing scleroderma renal crisis (SRC). Angiotensin covering enzyme (ACE) inhibitor therapy and calcium antagonist were effective for his renal condition. Although an association between SRC and anti-RNAP III antibody has already been reported in patients with diffuse cutaneous SSc (Dc SSc), this case indicates that SRC with hypetensive emergency with malignant nephrosclerosis can also be diagnosed on patients with Lc SSc patients by the examination of anti-RNAP III antibody.

Onion Skin Lesion of the Renal Small Artery in a Patient With Polymyositis and Hemolytic Uremic Syndrome

“…Immunofluorescence testing and electron microscopy did not reveal immune deposits. Lupus nephritis, antiphospholipid syndrome, or scleroderma renal crisis that can cause hemolytic uremic syndrome were excluded 1 , 2 , 3 This indicates that polymyositis is another disease that can cause hemolytic uremic syndrome. The only kidney biopsy image of a patient with hemolytic uremic syndrome due to polymyositis has been that of thrombotic microangiopathy with thrombus formation, as reported by Fukuda et al.…”

Scleroderma Renal Crisis Complicating Male-to-Female Transgender Hormonal Therapy in a Patient With Long-Standing and Stable Limited Cutaneous Systemic Sclerosis

“…Scleroderma renal crisis is almost always a complication of dcSSc rather than lcSSc, occurring within 5 years of disease onset, yet in this patient SRC occurred 7 years after lcSSc onset and a prolonged period of disease stability 8 . Anti-RNA polymerase III is associated with SRC and was positive in this case, although it is also uncommon among patients with lcSSc 8,9 . The remarkable acceleration of previously quiescent lcSSc in this patient, while on estradiol injections, raises the question of whether hormonal therapy contributed to this change in trajectory.…”

“…8 Anti-RNA polymerase III is associated with SRC and was positive in this case, although it is also uncommon among patients with lcSSc. 8,9 The remarkable acceleration of previously quiescent lcSSc in this patient, while on estradiol injections, raises the question of whether hormonal therapy contributed to this change in trajectory. A recent case series suggests a correlation between estrogen therapy and the development of SSc in 3 male-to-female transgender patients.…”

Renal Involvement in Systemic Sclerosis: An Update

“…Terras et al [44] and Stochmal et al [45] showed that the presence of anti-RNAP III antibodies in patients with SSc correlates with renal crisis and severe cutaneous involvement. Although an association between SRC and anti-RNAP III antibodies has already been reported in patients with dcSSc, Takada et al [46] recently reported a case of anti-RNAP III antibody-associated SRC in a patient with lcSSc.…”