Lung Pathology in Antiphospholipid Syndrome

Functional lesions of organs depend on the size of a diseased vessel and frequently require the use of intensive therapy methods. The commonest manifestation of antiphospholipid syndrome (APS) is deep vein thrombosis of the leg and pulmonary thromboembolism (PTE).

Objective: to estimate the frequency of lung lesions in primary APS (PAPS), secondary (in the presence of systemic lupus erythematosus (SLE)) and catastrophic APS and to assess a relationship between lung pathology and other clinical and laboratory manifestations of the disease.

Subjects and methods. The study covered 372 patients followed up at the Institute of Rheumatology, Russian Academy of Medical Sciences, since 1990, of whom 290 and 82 patients had SLE and PAPS, respectively. Among the 290 patients with SLE, there were 96 males and 194 females. At the moment of the study, the patients’ age was 31.2±11.1 years and the duration of the disease was 8.6±7.2 years. The group of patients with PAPS comprised 20 males and 62 females. Their mean age was 35.6±9.9 years and the duration of the disease was 11.9±8.5 years. Thrombotic events were verified only by instrumental studies. Lung pathology was instrumentally confirmed; all the patients underwent lung X-ray study, if required, scintigraphy and computed tomography.

Results. Lung lesion associated with the pathology of vessels was revealed in 28% of the examined patients (105/372). There were prevalent patients with PTE, followed by the development of lung infarcts, which was present in 96 (91%) of the 105 patients with pulmonary vascular pathology. Autopsy revealed pulmonary microangiopathy was in 12 patients, which was concurrent with focal pneumonia in 7 of them, with pneumonitis and exudative pleuritis in 5. Hemorrhagic alveolitis detected at autopsy in combination with occlusions of the pulmonary arterioles was in 3 patients who had been diagnosed as having thromboembolism of small branches of the pulmonary artery. Thrombosis of the pulmonary arterial trunk was detectable in 2 patients, both patients died from respiratory failure. All 105 patients with pulmonary vascular pathology had blood serological markers of APS. There was a combination of elevated levels of aKL and VA in 61% of cases and in 28.5% in the group of patients without pulmonary vascular pathology (OR = 3.92; [2.38-6.48]). The rate of vascular lung pathology increased in the presence of both blood aKL isotopes (IgG and IgM). The number of patients position in both aKL isotopes was 48% whereas in Group 2, that was 19.5% (OR = 3.76; [2.24-6.31]).

Conclusion. More than a fourth of the patients with SLE and PAPS have pulmonary vascular pathology. The spectrum of pulmonary vascular diseases in SLE is broad and varies from thrombosis of the pulmonary arterial trunk to occlusive vascular lesion of the microcirculatory bed of the lung, and it is associated with aFL.

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