A Schirmer test confirms inadequate tear production in this patient and anti-Ro/SSA antibodies are positive. Which of the following is the most likely explanation for this patient's neck mass?

Question

A Schirmer test confirms inadequate tear production in this patient and anti-Ro/SSA antibodies are positive.  Which of the following is the most likely explanation for this patient's neck mass?

Quizplus · Accepted Answer

Explanation: The Schirmer test is used to confirm the diagnosis of keratoconjunctivitis sicca. Filter paper is placed along the lower eyelid and wetting of the paper is measured after a defined period of time. Too little wetting of the paper is consistent with abnormal tear production. The presence of anti-Ro/SSA antibodies also helps to confirm the diagnosis of Sjogren's syndrome. The existence of a focal submandibular mass should raise concern for a B-cell non-Hodgkin's lymphoma in patients with Sjogren's syndrome. Sjogren's disease results in polyclonal B cell activation and infiltration of the salivary glands. This increased lymphocyte activation can go awry and result in the development of B-cell lymphoma in some patients. The lifetime risk of B-cell lymphoma is approximately 5% for Sjogren's syndrome patients - much higher than the risk for the general population. (Choice A) Adenocarcinoma from anywhere in the body can metastasize to neck lymph nodes, but this patient has no risk factors for adenocarcinoma, and her Sjogren's syndrome makes lymphoma much more likely. (Choice C) The chronic inflammation in Sjogren's syndrome is not associated with T cell activation, and therefore there is no increased risk of T cell lymphoma. (Choice D) Sjogren's syndrome is not associated with Hodgkin's lymphoma. Patients with Hodgkin's disease may have B symptoms consisting of fever, fatigue, and pruritus. (Choice E) Metastatic squamous cell carcinoma could present as a pathologic lymph node in the submandibular region. However, this is unlikely in this patient given the absence of alcohol or tobacco use. Educational objective: B lymphocyte activation occurs as a component of the chronic inflammation in patients with Sjogren's syndrome. This activation results in an increased risk of B cell lymphoma. Metastatic squamous cell carcinoma frequently spreads to lymph nodes in the head and neck but is usually associated with a history of tobacco or alcohol use. References: Hematologic manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspects. (http://www.ncbi.nlm.nih.gov/pubmed/19745687)

A Schirmer Test Confirms Inadequate Tear Production in This Patient