Sjögren syndrome: A case report

Abstract

Sjögren syndrome is a systemic autoimmune disorder that affects the exocrine glands. The authors present the case of a 50-year-old woman diagnosed with this syndrome on the basis of clinical and serological findings. Sonography of the major salivary glands revealed normal-sized glands with echo structures that were diffusely inhomogeneous due to the presence of multiple hypoechoic areas. Parenchymal vascularization was significantly increased on color Doppler imaging. These findings confirmed the diagnosis and revealed active disease manifested by glandular hypervascularization. This represents a typical case of Sjögren syndrome and illustrates the diagnostic value in these cases of salivary gland sonography, which is effective, noninvasive, and repeatable and provides accurate information on the vascularization status of the glands.Sommario La sindrome di Sjögren è una malattia autoimmune, sistemica, coinvolgente le ghiandole esocrine. Presentiamo il caso di una paziente, A.G., 50 anni, giunta alla nostra osservazione con diagnosi di Sindrome di Sjögren, sulla base della sintomatologia e degli esami ematochimici. Ecograficamente le ghiandole salivari maggiori erano nei limiti di norma per dimensioni, presentavano diffusa disomogeneità ecostrutturale bilateralmente per la presenza di multiple areole ipoecogene e vascolarizzazione parenchimale significativamente aumentata. L'ecografia confermava quindi la diagnosi ed evidenziava ipervascolarizzazione delle ghiandole, segno di attività della malattia. Abbiamo ritenuto il caso meritevole di segnalazione perché tipico e per sottolineare come la valutazione ecografica delle ghiandole salivari nella sindrome di Sjögren sia efficace, poco invasiva, facilmente ripetibile e permetta, un'accurata valutazione della vascolarizzazione ghiandolare. ª

Three-dimensional cone-beam computed tomographic sialography in the diagnosis and management of primary Sjögren syndrome: Report of 3 cases

“…Sjögren syndrome is a slowly progressive chronic autoimmune disorder characterized by symptoms of dry mouth and eyes, referred to as “sicca symptoms.” 1 2 In some cases, recurrent swelling in the parotid gland leads patients to consult multiple specialists without any symptomatic relief; furthermore, diagnosing the underlying cause of xerostomia that persists for >3 months is a challenge for dental professionals. 10 11 Early diagnosis is mandatory to achieve better clinical outcomes and prevent the complications associated with Sjögren syndrome.…”

“…The underlying disease process allowing the collection of contrast medium in form of dots or blobs is different; in Sjögren syndrome, the epithelium lining the intercalated ductule becomes weakened, whereas in sialadenitis, the acinus becomes dilated and eventually results in extravasation of contrast medium out of the duct. 1 2 The sialographic staging of sialectasia seen in Sjögren syndrome is based on the criteria of Rubin and Holt, 6 which divide it into 5 categories: normal, punctate (smooth oval ≤1 mm), globular (smooth oval 1–2 mm), cavitary (smooth oval >2 mm) and destructive (irregular >2 mm). Cavitary and destructive sialectasis are more prevalent in advanced cases, and punctate foci of calcifications in major salivary glands and multiple cystic or solid intraglandular masses in a miliary pattern are occasionally seen in Sjögren syndrome patients.…”

“…Its primary form involves exocrine glands, while its secondary form is associated with other autoimmune disorders. 1 The prevalence of Sjögren syndrome in the general population has been estimated to be 0.5% to 4.8%, it commonly occurs in middle-aged women between 40 and 50 years of age, and its male-to-female ratio has been reported to be 1 : 9. 2 The etiopathogenesis of this syndrome remains elusive, but the adaptive immune system has been documented to play an important role in the onset of Sjögren syndrome, as demonstrated by the presence of autoreactive T and B lymphocytes, abnormal regulation of apoptosis, production of autoantibodies to ribonucleoprotein particles (anti-SSA/Ro and anti-SSB/La), and lymphocytic infiltration of glandular tissues; these processes event-ually interfere with glandular function and result in impaired secretory activity, particularly of the salivary and lacrimal glands.…”

Renal Tubular Acidosis (RTA) due to Sjögren’s Syndrome Presenting as Hypokalemic Quadriparesis - A Case Report

“…2 Primary form involves the exocrine glands, with or without systemic involvement; there is also a secondary form, which is associated with other autoimmune diseases (rheumatoid arthritis, lupus erythematosus, scleroderma, inflammatory vascular and connective tissue diseases, etc.). 3 The renal manifestations are related to tubular dysfunction resulting from chronic interstitial nephritis and can exhibit as DRTA, proximal renal tubular acidosis, tubular proteinuria and nephrogenic diabetes insipidus. Hypokalemic paralysis rarely occurs as the first manifestation of a renal tubule disorder due to PSS.…”

“…Auto-antibodies (at least one)Anti-SSA (Ro) or Anti-SSB (La)For a primary Sjögren's diagnosis: Any 4 of the 6 criteria must include either item 4 (Histopathology) or 6 (Auto-antibodies) or any 3 of the 4 objective criteria(3,4,5,6).…”