Patients with Sjögren syndrome should be monitored for lymphadenopathy

Test your medicine knowledge with the MKSAP challenge, in partnership with the American College of Physicians.

A 63-year-old woman is evaluated during a follow-up visit for a 4-week history of fatigue; pain in the proximal interphalangeal joints, knees, and hips; and low-grade fever.

She has not had joint swelling, chest pain, or shortness of breath. Over the past 4 years, she has had progressive dryness of the eyes and mouth. She has a 5-month history of Raynaud phenomenon, which has been less symptomatic since beginning nifedipine 4 months ago.

On physical examination, temperature is 38.2 °C (100.8 °F), blood pressure is 125/72 mm Hg, pulse rate is 74/min, and respiration rate is 18/min. Cardiac examination is normal, and the lungs are clear. She has bilateral parotid gland enlargement, a firm 4-cm left axillary lymph node, and a shotty 0.3-cm left anterior cervical lymph node. Musculoskeletal examination reveals bilateral crepitus of the knees. There is no joint swelling.

Laboratory studies:

Hemoglobin	11.6 g/dL (116 g/L)
Leukocyte count	3400/µL (3.4 × 10⁹/L)
Platelet count	120,000/µL (120 × 10⁹/L)
Rheumatoid factor	76 U/mL (76 kU/L)
Antinuclear antibodies	Positive
Anti-Ro/SSA antibodies	Positive
Anti-La/SSB antibodies	Positive
Urinalysis	Normal
Blood cultures	No growth

A chest radiograph and mammogram are normal.

Which of the following is the next best step in this patient’s management?

A) Excisional axillary lymph node biopsy
B) Minor salivary gland biopsy
C) Prednisone
D) Transthoracic echocardiography

Answer and critique

The correct answer is A) Excisional axillary lymph node biopsy. This item is available to MKSAP 15 subscribers as item 79 in the Rheumatology section.

This patient’s symptoms of keratoconjunctivitis sicca (dry eyes and dry mouth); the presence of antinuclear, anti-Ro/SSA, and anti-La/SSB antibodies; and an elevated rheumatoid factor level are characteristic of primary Sjögren syndrome. Her fatigue, arthralgia, Raynaud phenomenon, and low-grade fever also are consistent with this condition. Sjögren syndrome also may occur secondary to another autoimmune disease, such as rheumatoid arthritis or systemic lupus erythematosus.

Patients with primary Sjögren syndrome have up to a 44-fold increased incidence of lymphoma, particularly non-Hodgkin lymphoma, and should be monitored closely for lymphadenopathy. This patient’s firm enlarged left axillary lymph node and pancytopenia particularly raise suspicion of lymphoma, and an excisional axillary lymph node biopsy is the most appropriate next step in this patient’s management.

A minor (labial) salivary gland biopsy would help to confirm the diagnosis of Sjögren syndrome and would reveal increased lymphocytic infiltration. Because this patient has clinical evidence of Sjögren syndrome, salivary gland biopsy is not necessary; furthermore, this study would not help to evaluate this patient’s enlarged axillary lymph node.

Prednisone may help to treat constitutional symptoms associated with Sjögren syndrome but would not address this patient’s more urgent enlarged left axillary lymph node. In addition, her constitutional symptoms may be manifestations of malignancy and not Sjögren syndrome.

Infective endocarditis may manifest as fever, fatigue, and lymphadenopathy, and transthoracic echocardiography can help to diagnose this condition. However, infective endocarditis is unlikely in a patient with no cardiac abnormalities; negative blood culture results; and an absence of associated vascular phenomena or risk factors for this condition. Therefore, transthoracic echocardiography would not be warranted in this patient.

Key Point

Patients with primary Sjögren syndrome have up to a 44-fold increased incidence of lymphoma and should be monitored closely for lymphadenopathy.

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