clinical characteristics of other extraglandular manifestations of primary Sjogrens Syndrome

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clinical characteristics of other extraglandular manifestations of primary Sjogrens Syndrome

• Lung manifestations: includes xerotrachea/xerobronchitis, nonspecific interstitial pneumonitis, lymphocytic interstitial pneumonitis, usual interstitial pneumonitis, bronchiolitis (constrictive), and bronchial-associated lymphoid tissue lymphoma. Due to thick secretions and recurrent pneumonias, patients can develop bronchiectasis. Pearl: Consider SS in any patient with unexplained lung disease and a positive ANA.

• Renal disease: includes type I distal renal tubular acidosis (10%) that rarely can lead to severe potassium wasting and muscle paralysis, tubular interstitial nephritis (<5%), glomerulonephritis, and nephrogenic diabetes insipidus

• GI: includes a higher prevalence of celiac disease (both diseases share similar genetics of HLA-DRB1∗0301 and HLA-DQB1∗0201). Suspect in patients with diarrhea and vitamin D deficiency. Primary biliary cholangitis (5%), autoimmune hepatitis, and recurrent pancreatitis (<5%) have also been described.

• Vasculitis: cutaneous vasculitis (palpable purpura) is most common, whereas necrotizing vasculitis (similar to polyarteritis nodosa) or cryoglobulinemia in the absence of hepatitis C is infrequent.

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