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Table 1 Summary of reported SCC-TMA cases in overlap syndrome

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

 

Current case

Case 1

Case 2

Case 3

Case 4

Case 5

Author

Hongrui Dong

Jordana Cheta

Greenberg

Yuki Nanke

Carlos Quereda

Year

2020

2017

2001

2000

1991

Age (year)

21

61

50

64

33

47

Gender

Female

Male

Female

Female

Female

Female

CTD

SCC-SLE-PM

SCC-SLE

MCTD

SCC-DM

SCC-SLE-RA

SCC-PM

CTD History

0y

5y

10y

0y

7y

2y

Other organs

CHF ILD

DAH

ILD

Renal pathology

Stenosis and ischemia in glomerular capillary loops; moderately thickened vessels; edema endothelial, intimal thickening and occlusion in arteries.

Mucinous edema and “onion bulbs” in interlobular arteries;

widened loose layer of glomerular basement membrane.

1/9 glomerulus had a thrombus within an afferent arteriole; moderately thickened vessels; multiple red cell casts and dilated tubules.

Severe thrombotic and mesangiolysis in glomerular; multiple sclerosis or occluded vascular lumen with thrombi; focal tubular necrosis.

Multiple thrombi in afferent arterioles of glomeruli; fibrous and edematous arteries.

Multiple thrombi in glomerular capillary loops; endothelial proliferation, intimal thickening and thrombi occlusion in arteries.

Treatment

CS, RASI, HD-PD, RTX

CS, CTX, RASI

CS, RASI, HD

CS, RASI, HD

CS, CTX, RASI HD, PE

CTX, RASI, HD-PD

Prognosis

Normal renal function

Death

Normal renal function

Dependent on dialysis

Death

Dependent on dialysis

  1. Note: CHF congestive heart failure; CS corticosteroid; CTD connective tissue disease; CTX cyclophosphamide; DAH diffuse alveolar hemorrhage; DM dermatomyositis; HD hemodialysis; ILD interstitial lung disease; MTCD mixed connective tissue disease; PD peritoneal dialysis; PE plasma exchange; PM polymyositis; RA rheumatoid arthritis; RASI renin-angiotensin-aldosterone system inhibitor; RTX rituximab; SCC systemic sclerosis; SLE systemic lupus erythematosus; y year