Pyoderma gangrenosum, subcorneal pustular dermatosis, IgA paraproteinaemia and IgG antiepithelial antibodies.

OBJECTIVES

We aimed to assess the defensive profile of primary Sjögren's syndrome (SS) patients and to investigate the independent associations of psychological distress and personality variables with health-related quality of life (HRQOL).

METHODS

In 40 primary SS patients we assessed psychological distress (SCL-90-R), ego defense mechanisms (Defense Style Questionnaire), hostility features (HDHQ) and HRQOL (WHOQOL-BREF). Fifty-six patients with Systemic Lupus Erythematosous (SLE) and 80 healthy participants matched for age and sex served as controls.

RESULTS

Primary SS patients presented higher rates of general psychological distress compared to SLE and healthy participants. Symptoms of somatisation were more prominent in SS than SLE or healthy controls. SS patients presented less use of humour defense and more help-rejecting complains and delusional guilt hostility, compared to controls. Primary SS patients' HRQOL was more impaired than healthy participants and comparable to SLE. Psychological distress was a constant independent correlate of SS patients' HRQOL, while less use of humour (p<0.001) and higher rates of delusional guilt (p=0.032) were also significantly associated with Physical HRQOL independently of psychological distress; more use of schizoid fantasy was also independently associated with impaired Environment HRQOL (p=0.005).

CONCLUSIONS

Primary SS patients exhibit several specific psychological difficulties in adaptation to life stressors, and clinicians and consultation-liaison psychiatrists, apart from the early assessment and treatment of psychological distress and somatisation symptoms, should consider the patients' underlying defensive profile and coping capacities, since such personality traits, although usually underestimated, are also independently associated with the disease outcome.