Thrombotic Microangiopathy TMA

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TMA is the characteristic histologic lesion of the microvascu-lature in APS-related nephropathy, affecting glomerular capillaries, afferent arterioles, and interlobular arteries (Fig. 7.10). Of course, it is not pathognomonic of APS, as there is a wide range of conditions that present the same histological appearance (Table 7.6).

Depending on the degree and extension of damage, patients could have isolated hypertension (mild to severe), proteinuria

Fig. 7.9. Primary APS with giant skin ulcer on the left leg refractory to anticoagulant and fibrinolytic treatment, from Khamashta 2000, with kind permission from Springer.
Table 7.5. Renal vascular involvement in APS.

Vascular lesion Renal artery lesions (thrombosis/occlusion/ stenosis?)

Clinical consequences Renovascular hypertension (severe) Renal infarcts (silent, painful, hematuria)

Glomerular capillary thrombosis leading to glomerular sclerosis (studied mainly in SLE patients)

Increased likelihood of renal insufficiency

Can be observed in up to 40% of aPL negative patients

Renal Thrombotic microangiopathy with/ without cortical necrosis

Systemic hypertension (usually severe) Renal failure (mild to end stage) Proteinuria (mild to nephrotic range) Cortical atrophy

Renal vein thrombosis (uni or bilateral)

Renal failure (if bilateral compromise)

(mild to nephrotic range), and renal failure including cortical necrosis (Fig. 7.11). In fact, APS should be considered in the differential diagnosis of systemic hypertension as APS-related TMA may cause isolated hypertension without significant

  1. 7.10. Severe and advanced glomerular thrombotic microan-giopathy. Capillary lumina are occluded by severe mesangiolysis and deposition of heterogeneous subendothelial material, leading to a segmental "double contour" aspect (PAS), from Khamashta 2000, with kind permission from Springer.
  2. 7.10. Severe and advanced glomerular thrombotic microan-giopathy. Capillary lumina are occluded by severe mesangiolysis and deposition of heterogeneous subendothelial material, leading to a segmental "double contour" aspect (PAS), from Khamashta 2000, with kind permission from Springer.

Table 7.6. Other conditions associated with TMA.

Thrombotic thrombocytopenic purpura (TTP) Hemolytic uremic syndrome (HUS) Postpartum renal failure Pre-eclampsia/eclampsia Scleroderma

Malignant arterial hypertension Oral contraceptives

Renal transplantation / allograft rejection Cyclosporine A toxicity Chemotherapy renal impartment, as well as in the differential diagnosis of renal damage in patients with SLE and positive aPL.

In chronic cases, fibrosis and focal atrophy, as well as arterial and arteriolar fibromuscular hyperplasia, could be found (Fig. 7.12).

Obsolescent Glomeruli
  1. 7.11. Chronic cortical lesions. There are obsolescent sclerotic glomeruli and a hypoperfused glomerulus with a wide Bowman's space and retracted capillaries. On one of its sides there is a small arteriole with a recanalized thrombus and two lumina. On the other side there are two completely occluded arterioles showing thrombosis, recanalization, and refibrosis. A tortuous arteriole with slightly cellular subendothelial fibrosis is also seen (PAS), from Khamashta 2000, with kind permission from Springer.
  2. 7.11. Chronic cortical lesions. There are obsolescent sclerotic glomeruli and a hypoperfused glomerulus with a wide Bowman's space and retracted capillaries. On one of its sides there is a small arteriole with a recanalized thrombus and two lumina. On the other side there are two completely occluded arterioles showing thrombosis, recanalization, and refibrosis. A tortuous arteriole with slightly cellular subendothelial fibrosis is also seen (PAS), from Khamashta 2000, with kind permission from Springer.

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