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Patient Case: Sjögren’s Syndrome in Sub-Saharan Africa

Patient Case: Sjögren’s Syndrome in Sub-Saharan Africa

Discussion

A 45-year-old woman presents to a rural clinic with complaints of persistent dry mouth and dry eyes for the past two years. She reports difficulty swallowing dry foods and a constant feeling of grittiness in her eyes. Over the last six months, she has experienced joint pain, especially in her wrists and knees, along with fatigue that interferes with her daily activities.

On examination, her parotid glands appear mildly swollen, and a Schirmer’s test confirms decreased tear production. Blood tests reveal positive anti-SSA and anti-SSB antibodies, supporting the diagnosis of primary Sjögren’s syndrome. Due to limited access to specialty care, she is managed with artificial tears, saliva substitutes, and hydroxychloroquine for her joint symptoms. She is advised on oral hygiene to prevent dental complications and scheduled for follow-up at a tertiary center for further evaluation.

Questions
1. What is the most likely diagnosis in this patient presenting with dry mouth, dry eyes, joint pain, and fatigue?

A) Rheumatoid arthritis

B) Sjögren’s syndrome

C) Systemic lupus erythematosus

D) Scleroderma

2. Which diagnostic test result is most supportive of the suspected diagnosis?

A) Positive rheumatoid factor (RF)

B) Elevated ESR and CRP

C) Positive anti-SSA and anti-SSB antibodies

D) Low complement levels

3. What is the primary treatment approach for the patient's dry eyes and dry mouth?

A) Systemic corticosteroids

B) Artificial tears and saliva substitutes

C) Methotrexate

D) Nonsteroidal anti-inflammatory drugs (NSAIDs)

4. What is a major complication this patient is at increased risk for due to her condition?

A) Pulmonary fibrosis

B) Lymphoma

C) Myocarditis

D) Deep vein thrombosis

Reveal answers

Answers

1 - B) Sjögren’s syndrome

Sjögren’s syndrome is an autoimmune disorder primarily affecting the exocrine glands, leading to dry eyes, dry mouth, joint pain, and fatigue. Other conditions, such as rheumatoid arthritis and lupus, may have overlapping features but do not primarily cause sicca symptoms.

2 - C) Positive anti-SSA and anti-SSB antibodies

Anti-SSA (Ro) and anti-SSB (La) antibodies are the most specific markers for Sjögren’s syndrome. While RF and ESR/CRP may be elevated, they are not diagnostic. Low complement levels can be seen in severe or secondary cases but are not a primary diagnostic feature.

3 - B) Artificial tears and saliva substitutes

Management of Sjögren’s syndrome focuses on symptomatic relief. Artificial tears and saliva substitutes help manage dryness, while hydroxychloroquine may be used for systemic symptoms. Systemic corticosteroids are reserved for severe extraglandular involvement.

4 - B) Lymphoma

Patients with Sjögren’s syndrome have a significantly increased risk of developing non-Hodgkin’s lymphoma due to chronic B-cell activation. Symptoms such as persistent glandular swelling should raise suspicion for malignancy.

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