The expanding spectrum of renal diseases associated with antiphospholipid syndrome.
BACKGROUND
The association of thrombotic events and/or pregnancy complications with circulating antiphospholipid antibodies defines antiphospholipid syndrome (APS). In previous reports, renal involvement in APS consisted mainly of thrombotic vascular complications involving large vessels or intrarenal small-sized vessels (APS nephropathy). We report 9 cases of glomerulonephritis associated with APS. These cases are characterized by predominant pathological features distinct from vascular APS nephropathy.


METHODS
We reviewed consecutive renal biopsies examined in 2 French university hospitals between 1980 and 2002 and identified renal biopsies performed in patients with primary APS.


RESULTS
We identified 29 biopsies performed in patients with APS. Twenty biopsies showed characteristic features of APS nephropathy. In 9 cases, predominant pathological features distinct from vascular APS nephropathy were noted: membranous nephropathy (3 cases), minimal change disease/focal segmental glomerulosclerosis (3 cases), mesangial C3 nephropathy (2 cases), and pauci-immune crescentic glomerulonephritis (1 case). In 7 cases, the presentation of renal symptoms was subacute or chronic. Two patients experienced episodes of acute renal failure. At referral, median creatinine clearance was 50 mL/min (0.83 mL/s) (range, 18 to 117 mL/min [0.30 to 1.95 mL/s]). Proteinuria was noted in all cases (range, 1.5 to 15 g/d), with nephrotic syndrome in 4 cases. Lupus anticoagulant was present in all cases, and anticardiolipin antibodies, in 8 cases. Anti-DNA antibodies repeatedly were negative in all cases. Treatment consisted of antihypertensive therapy (6 cases), anticoagulant drugs (5 cases), steroids (4 cases), and antiplatelet drugs (3 cases). At last follow-up, renal function remained stable in 7 patients. Of 2 patients presenting with acute renal failure, 1 patient recovered normal renal function, whereas the other patient progressed to end-stage renal failure.


CONCLUSIONS
The cases reported here represent a new aspect of the expanding spectrum of renal diseases encountered in association with APS.
Fakhouri
Fadi
F
Department of Nephrology, AP-HP, Hôpital Necker, Paris, France. fakhouri@necker.fr
Noël
Laure-Hélène
LH
Zuber
Julien
J
Beaufils
Hélène
H
Martinez
Frank
F
Lebon
Pierre
P
Papo
Thomas
T
Chauveau
Dominique
D
Bletry
Olivier
O
Grünfeld
Jean-Pierre
JP
Piette
Jean-Charles
JC
Lesavre
Philippe
P
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D001706:Biopsy; D001798:Blood Proteins / Q000032:analysis; D005260:Female; D005602:France / Q000453:epidemiology; D005921:Glomerulonephritis / Q000453:epidemiology / Q000209:etiology; D015432:Glomerulonephritis, Membranoproliferative / Q000453:epidemiology / Q000209:etiology; D015433:Glomerulonephritis, Membranous / Q000453:epidemiology / Q000209:etiology; D005923:Glomerulosclerosis, Focal Segmental / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000188:drug therapy / Q000209:etiology; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000209:etiology; D008297:Male; D009402:Nephrosis, Lipoid / Q000453:epidemiology / Q000209:etiology; D009404:Nephrotic Syndrome / Q000453:epidemiology / Q000209:etiology; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D012189:Retrospective Studies; D019851:Thrombophilia / Q000188:drug therapy / Q000209:etiology
D000305:Adrenal Cortex Hormones; D000925:Anticoagulants; D000959:Antihypertensive Agents; D001323:Autoantibodies; D001798:Blood Proteins; D010975:Platelet Aggregation Inhibitors