Clinical manifestations of APS-nephropathy in primary antiphospholipid syndrome


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Abstract

Aim. To elicit clinical features of nephropathy associated with antiphospholipid syndrome (APSN) in
patients with primary antiphospholipid syndrome (PAPS).
Material and methods. The analysis of clinical characteristics and course of APSN has covered 24
patients with PAPS (16 females and 8 males, mean age 34.3 years). Renal damage was represented by
arterial hypertension (AH), urinary syndrome, functional decline. All the patients were tested for anticardiolipin
antibodies and/or lupus anticoagulant. Renal biopsy was made in 7patients.
Results. PAPS patients developed renal affection in the onset of APS or within the first 5 years of its
course. In the majority of patients APSN combined with abnormalities of CNS, heart and skin. Arterial/arteriolar
thromboses prevailed. APSN manifested with: AH (n = 23, severe AH in 11), abnormal
renal fdtration (n = 17, creatinine rise in 8), urinary syndrome with proteinuria (n = 23, in 14 with
hematuria). The following clinical variants of APSN were proposed: urinary syndrome with AH
(n = 16; 67%), acute nephritic syndrome (n = 7; 29%), nephrotic syndrome (n = 1). Morphological
studies of biopsies from APSN patients have revealed sclerotic changes, thrombotic microangiopathy,
nonspecific alterations in the glomeruli.
Conclusion. APSN is a variant of microvascular renal affection caused by thrombotic processes in intraorgan
microcirculation, ft is an early clinical marker of APS. Clinically, APSN manifests with vascular
renal affection, the earliest symptom being inhibition of glomerular filtration. Clinical combinations
of the symptoms allow to distinguish variant of APSN suggesting the existence of acute and
chronic APSN. Combination of APSN with affection of the CNS, heart and skin points to a special
PAPS subtype characterized by generalized ischemic damage to the organs as a result of intraorganic
arterial and/or arteriolar thromboses.

References

  1. Hughes G. R. V., Harris Е. N., Gharavi A. Е. The anticardiolipin syndrome. J. Rheumatol. 1986; 13: 486-489.
  2. Piette J. C., Kleinknecht D., Bach J.-F. Renal manifestation in the antiphospholipid syndrome. In: Asherson R., Cervera R.,Piette J. C, Schoenfeld J., eds. The antiphospholipid syndrome. Boca Raton: CRC Press; 1996. 169-181.
  3. Nochy D., Daugas E., Droz D. et al. The intrarenal vascular lesions associated with primary antiphospholipid syndrome. J.Am. Soc. Nephrol. 1999; 10: 507-518.
  4. Daugas E., Nochy D., Thi Huong D. L. et al. Antiphospholipid syndrome nephropathy in systemic lupus erythematosus. Ibid. 2002; 13: 42-52.
  5. Amiga M.-C., Garcia-Torres R., Robles M. et al. Renal involvement in primary antiphospholipid syndrome. J. Rheumatol. 1992; 19: 1181-1185.
  6. Kincaid-Smith P., Fairley K. F., Kloss M. Lupus anticoagulant associated with renal thrombotic microangiopathy and preg- nancy-related renal failure. Quart. J. Med. 1988; 69: 795-815.
  7. Nochy D., Daugas E., Thi Huong D. L. et al. Kidney involvement in the antiphospholipid syndrome. J. Autoimmun. 2000; 15: 127-132.
  8. Мухин Н. А., Лысенко Л. В., Козловская Н. Л. и др. Поражение почек при антифосфолипидном синдроме. Врач 2002; 6: 13-18.
  9. Козловская Н. Л., Тареева И. E., Камышова Е. С. и др. Поражение почек при первичном антифосфолипидном синдроме. Тер. арх. 2001; 6: 25-33.
  10. Moss К. Е., Isenberg D. A. Comparison of renal disease and outcome in patients with primary antiphospholipid syndrome, antiphospholipid syndrome secondary to systemic lupus erythematosus (SLE) and SLE alone. J. Rheumatol. 2001; 40: 863-867.
  11. Jesus N. R., Levy R. A., Albuquerque E. M. N., Porto L. C. M. S. Preclampsia as the first manifeststion of APS. Lupus 2002; 11: 612.
  12. Piette J. С. Venosus vs arterial/arteriolar APS subsets. Ibid. 634.
  13. Branch D. W., Silver R. M. Criteria for antiphospholipid syndrome: early pregnancy loss, fetal loss or recurrent pregnancy loss? Ibid. 1996; 5: 409-413.
  14. Козловская Н. Л., Швецов М. Ю., Козловская Л. В. и др. Поражение почек у больных системной красной волчанкой с антифосфолипидным синдромом и первичным антифосфолипидным синдромом. В кн.: Мухин Н. А. (ред.) Успехи нефрологии. М.: ММА им И. М. Сеченова; 2001. 227-236.
  15. Levy Y., George J., Ziporen L. et al. Massive proteinuria as a main manifestation of primary antiphospholipid syndrome. Pathology 1998; 66 (1): 49-52.
  16. Vianelli M., Cavazzana I., Napodano P. et al. Renal involvement in primary and secondary APS. Lupus 2002; 11: 620.

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