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Are the cutaneous manifestations in patients with primary antiphospholipid syndrome a marker for predicting lung manifestations?

1, 2, 3, 4, 5, 6

 

  1. Clinic for Pulmonology, Clinical Center of Serbia, and School of Medicine, University of Belgrade, Serbia.
  2. Internal medicine, “Bezanijska Kosa”, University Medical Center, Belgrade, Serbia.
  3. General Hospital Sabac, Serbia.
  4. Regional Medical Center “Južni Banat”, Pančevo, Serbia.
  5. General Hospital Sremska Mitrovica, Serbia.
  6. School of Medicine, University of Belgrade; and Internal Medicine, “Bezanijska Kosa”, University Medical Center, Belgrade, Serbia.

CER10326
2018 Vol.36, N°1
PI 0056, PF 0061
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PMID: 28770705 [PubMed]

Received: 14/02/2017
Accepted : 18/04/2017
In Press: 17/07/2017
Published: 05/02/2018

Abstract

OBJECTIVES:
The aim of this study was to investigate association between pulmonary and skin manifestations in a large group of patients with primary antiphospholipid syndrome (PAPS) as well as their connection with antiphospholipid antibodies.
METHODS:
Our prospective study comprises of 390 patients with primary APS. Antiphospholipid antibody (aPL) analysis included detection of aCL (IgG/IgM), ß2GPI (IgG/IgM) and LA. Distinct pulmonary and skin associations were determined, as well as their associations with aPL.
RESULTS:
In PAPS patients the presence of LA was more common in PTE (p=0.005) and in pulmonary microthrombosis (p=0.003). We revealed statistical significance considering the presence of aCL IgM and pulmonary microthrombosis (p=0.05). Skin ulcerations correlated with positive titres aCL IgM and ß2 GPI IgM (p=0.03 and 0.04, respectively), while pseudovasculitis correlated with positive titres ß2 GPI IgM (p=0.02). PAPS patients were more more likely to develop pulmonary thromboembolisam if they had livedo reticularis (p=0.005), skin ulcerations (p=0.007), pseudovasculitic lesions (p=0.01), superficial cutaneous necrosis (p=0.005), and digital gangrene (p=0.02). Patients were also more prone to pulmonary microthrombosis if they already had livedo reticularis (p=0.03), skin ulcerations (p=0.007), pseudovasculitic lesions (p=0.05), superficial cutaneous necrosis (p=0.006), and digital gangrene (p=0.02).
CONCLUSIONS:
There is strong link between some pulmonary and skin manifestations in PAPS patients, suggesting complexity and evolutionary nature of APS. The presence of skin manifestations may be a high risk factor for several types of serious pulmonary manifestations in PAPS. Certain aPL types are associated with distinct pulmonary and skin manifestation, suggesting their predictive role.

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