How do you manage patients with systemic sclerosis and chronic thrombotic microangiopathy on renal biopsy, but no other evidence of scleroderma renal crisis?

Very good question. Disclaimer 1^st - I am not a nephrologist or nephropathologist so this really requires putting many heads together!

Thrombotic microangiopathies (TMA) are clinical syndromes defined by the presence of hemolytic anemia, thrombocytopenia, and organ damage from micro thrombosis in capillaries and small arteries. TMA is most often a feature of TTP (severe ADAMTS13 deficiency-genetic or acquired) and HUS (diarrheal Shiga-toxin), but can also be seen in secondary TTP/HUS (eclampsia/...