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Gazzetta Medica Italiana - Archivio per le Scienze Mediche 2020 March;179(3):187-92

DOI: 10.23736/S0393-3660.19.04097-X


lingua: Inglese

Sarcoidosis presenting as sicca syndrome

Joana A. PEREIRA 1 , Ana M. RIBEIRO 1, Joanne M. LOPES 2, Patrícia L. LOURENÇO 1

1 Department of Internal Medicine, Centro Hospitalare Universitario S. João, Porto, Portugal; 2 Department of Pathology Anatomy, Hospital of São João, Porto, Portugal

Sarcoidosis is a disease of unclear etiology that can affect multiple organs. It is a condition in which there is deposition of noncaseating granulomas in the affected structures. Lung is the most commonly affected organ, but in up to a third of the patients there are also extra-pulmonary manifestations. The etiologic investigation of sicca symptoms can be challenging. Clinicians more commonly associate them to other diseases - mainly Sjögren Syndrome - but they may also be, in rare cases, the presentation form of Sarcoidosis when salivary glands are affected. Visceral involvement by the granulomatous process can coexist with other manifestations of the disorder, with a wide range of clinic, laboratory and radiographic presentation. Liver is one of the extra-pulmonary structures most commonly impaired although the majority of patients are asymptomatic. We report the case of a patient presenting with sicca symptoms and retromandibular tumefactions. The investigation revealed elevated angiotensin-converting enzyme, mild elevation of liver enzymes, increased urinary calcium excretion and negative anti-SSA/Ro antibodies. The histological data of the parotid gland and liver biopsies were compatible with a granulomatous process and the research for Mycobacterium tuberculosis was negative. Thoracic computed tomography showed mediastinal, hilar and subcarinal lymph nodes. The final diagnosis of liver, exocrine glands and pulmonary (stage 1) Sarcoidosis was established.

KEY WORDS: Sarcoidosis; Sjogren’s Syndrome; Lung

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