Primary Sjögren syndrome and pulmonary cysts disease: case report

Abstract

Context: Sjögren's syndrome (SS) is an inflammatory, chronic autoimmune disease of unknown etiology, which is characterized by infiltration of glandular and extraglandular tissue by lymphocytes and plasma cells. It presents glandu-lar manifestations (xerophthalmia and xerostomia), as extraglandular (50%). Within these, there are pulmonary manifestations (11%), the most common manifestation being lymphocytic interstitial disease followed by cystic lung dis-ease (25%). Its manifestations often precede the glandular manifestations so that the diagnosis of it is confused or delayed.

Case presentation: A 71-year-old female patient with a history of high blood pressure and hypothyroidism. Hospitalized for presenting asthenia, dry cough and dyspnea to the medium efforts. In the review of all systems, she referred xerophthalmia and xerostomia. The following exams were carried out: Rx thor-ax: rounded diffuse opacities in both pulmonary fields, is complemented with simple chest Tomography: Multiple pre-dominant cystic lesions in lower lobes and nodular calcifications. It was decided to expand the studies to determine etiology and see if it is related to its xerophthalmia and xerostomia characteris-tic of SS. Immunochemical tests were performed. The results: anti-SSA / Ro 200 U / mL (< 20 EU / mL); anti SSB / 84.4U / mL (< 20 EU / mL); antinuclear antibodies with fine granular pattern 1/160 (≤ 1/40) Rheumatoid factor 49.9UI / mL (< 20 IU / mL). With the suspicion of Sjogren's syndrome, parotid sialog-raphy is performed, obstruction is evidenced and chronic inflammatory pro-cesses of parotid ducts. Lower lip biopsy: Mucosa lined by squamous epitheli-um with intercellular edema and exocytosis of small lymphocytes. The stroma contains mild scattered lymphomononuclear infiltrate characteristic of SS. Con-firmed diagnosis of SS managed with corticosteroids and specific treatment of eye and dry mouth.