AI Article Synopsis

  • Pulmonary embolism is the most common symptom of primary antiphospholipid syndrome (PAPS), but PAPS can also cause respiratory issues through non-thrombotic means.
  • A case study is presented that shows PAPS-related diffuse alveolar hemorrhage (DAH), highlighting the lack of guidelines for treating this condition.
  • In the case discussed, initial treatments with cyclophosphamide or mycophenolate mofetil and low dose prednisone were ineffective, likely due to infections, but adding monthly intravenous immunoglobulin helped control the disease.

Article Abstract

Pulmonary embolism is the most common pulmonary manifestation of primary antiphospholipid syndrome (PAPS). However, PAPS may manifest in the respiratory system also due to non-thrombotic processes. In the following paper we present a case of PAPS-related diffuse alveolar hemorrhage (DAH). Because of sparse literature and a lack of randomized controlled trials, there are currently no recommendations regarding the optimal choice of steroid-sparing agent in treating PAPS-related DAH. In our patient, treatment with cyclophosphamide or mycophenolate mofetil along with low dose prednisone was ineffective, partially because of infectious complications, whereas addition of monthly intravenous immunoglobulin to mycophenolate mofetil and prednisone, appears to control the disease.

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http://dx.doi.org/10.5603/ARM.2018.0020DOI Listing

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