Primary Sjögren's syndrome (pSS) is a systemic autoimmune disorder that predominantly affects exocrine glands leading to eye and mouth dryness, fatigue and pain. However, up to 40% of the affected patients will develop extraglandular involvement during the disease course attributed to lymphocytic infiltration of affected organs including the lungs, kidneys and liver. In addition, immune complex mediated manifestations might occur as purpura, peripheral neuropathy or glomerulonephritis.
It is not uncommon for this disease entity to present itself as a diagnostic dilemma and to pose therapeutic challenges especially in cases of multiple organ involvement. To underline the complexity of this condition, we will present two difficult cases outlining the disease course right from the initial presentation and ending with the most recent clinical status. This will be followed by a detailed discussion pertaining to the diagnostic and therapeutic challenges as well as the respective complications that may arise.
The first case involves a 78 year old female patient, who developed severe pulmonary involvement in the form of nonspecific interstitial pneumonia (NSIP) and pulmonary hypertension. The second case presentation will focus on a 71 year old female patient developing severe neurological manifestations with progressive polyneuropathy leading to tetraparesis of the extremities.
Both cases should raise awareness to the wide array of clinical presentations, their ambiguity especially in the lack of histological evidence, the sequelae that follow and last but not least, the degree of associated morbidity not only related to the pathophysiology of this condition but also to that resulting from immunosuppressive therapy.
Disclosure of Interest None declared