Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors

Table 1

Summary of case reports of scleroderma renal crisis (SRC) in mixed connective tissue disease (MCTD).

Case report	Sex/age	Clinical background	Pathological features	Treatment	Outcome
Our case	Female/30	History of MCTD presented with vomiting received aggressive fluid resuscitation for suspected dehydration resulting in hypertensive emergency, pulmonary edema, and AKI	Biopsy revealed TMA type changes within 3 interlobular arteries. The changes compatible with malignant HTN or SRC. 1^st interlobular artery shows moderate edematous mucoid intimal thickening, the 2^nd shows intimal fibrinoid change, and the 3^rd shows moderate to marked intimal thickening with intimal fibrosis and mild edema	Captopril	Responded to treatment
Cheta et al. [5]	Female/54	Patient presented with shortness of breath, chest pain, Raynaud’s phenomenon, and AKI. Diagnosed with a MCTD flare, renal failure, and pneumonia	3/7 intraglomerular thrombi and moderately thickened vessels. Multiple red cell casts within tubular lumina with mild interstitial fibrosis. No evidence of SRC or HUS type TMA	Captopril, MMF, plasma exchange, steroid, HD	Responded to treatment (Cr 1.7 mg/dL)
Vij et al. [22]	Male/21	Oliguria, scleroderma facies, hypertension, and AKI	Bloodless glomeruli, thickening of glomerular capillary walls, interlobular vessels fibrointimal hyperplasia with obliteration of capillary lumen, tubular injury, and interstitial edema	Plasma exchange, HD	HD dependent
Khan et al. [23]	Female/36	Hx of Raynaud’s phenomenon, blurry vision, arthralgias, and oliguric renal failure	14 glomeruli were seen which showed nonimmune complex-mediated disease process, ischemic collapse with fibrinoid necrosis. Tubules revealed patchy degeneration with interstitial edema and hyaline casts	Captopril	HD dependent (Cr in range of 2.5–3.0 mg/dl)
Khalil et al. [12]	Male/44	Hypertension, dyspnea, vomiting, Raynaud’s phenomenon, skin tightening, and AKI.	2/11 sclerosed glomeruli, remaining glomeruli showing mild to severe capillary collapse. Intimal thickening of blood vessel wall.	HD, captopril	HD dependent (Cr 7.7 mg/dL)
Celikbilek et al. [20]	Female/30	History of sausage-like swellings, Raynaud’s phenomenon. Renal dysfunction and pulmonary involvement developed following abortion.	7/12 glomeruli with global sclerosis. Interstitial fibrosis and dense mononuclear cell infiltration. Tubular atrophy. Arterial walls with prominent thickening and hyalinization.	Enalapril, steroids, CTX.	Responded to treatment
Anderson and Vasko [28]	Case 1: female/64; Case 2: male/45	Both cases had features of Raynaud’s phenomenon and pulmonary HTN. SRC was provoked by steroids in case 1 and by CHF in case 2.	Case 2: kidney biopsy at autopsy shows renal interlobular arteries and arterioles with edematous, concentric, myxoid intimal proliferation, and thickening almost totally obliterating lumen in a few vessels. These findings were in accordance with SRC.	Enalapril	Response to treatment in both cases (Cr12.03,Cr21.35 mg/dL).
Greenberg and Amato [27]	Female/64	Inflammatory myopathy and bilateral carpal tunnel syndrome who developed AKI following steroid therapy.	Active and severe TMA with extensive mesangiolysis and glomerular capillary wall remodeling with double contours in many glomeruli. Severe arterial and arteriolar sclerosis with fibrin thrombi occlusion.	ACEi, HD	HD dependent (Cr 7.2 mg/dL)
Satoh et al. [6]	Female/47	Raynaud’s phenomenon with swollen fingers, sclerodactyly, lymphadenopathy who developed accelerated HTN, AKI and MAHA.	22 glomeruli showed mild ischemic changes. Prominent vascular changes in 2 small arteries, 1/2 with complete occlusion by thrombi and the other with mild intimal proliferation. IF showed faint staining of IgM in the glomerular mesangium.	PSL, PGs, ACEi	Responded to treatment (Cr 1.0 mg/dL)

MCTD, mixed connective tissue disease; SRC, scleroderma renal crisis; AKI, acute kidney injury, Cr, creatinine; HUS, hemolytic-uremic syndrome; TMA, thrombotic microangiopathy; TTP, thrombotic thrombocytopenic purpura; HTN, hypertension; CHF, congestive heart failure; MAHA, microangiopathic hemolytic anemia; IF, immunofluorescence; HD, hemodialysis; MMF, mycophenolate mofetil; ACEi, angiotensin-converting enzyme inhibitor; CTX, cyclophosphamide; PSL, prednisolone; PGs, prostaglandins.