APLAR Grand Round Blackboard 2023

Step 1 of 2

Question 1

A 38-year-old woman with primary Sjogren’s syndrome develops severe cutaneous vasculitis refractory to the initial treatment of glucocorticoids. What should be the next step in managing her condition?
Question 1
- A. Tumor necrosis factor (TNF) inhibitors
- B. Anifrolumab
- C. Rituximab
- D. Belimumab
Answer with a detailed explanation
C. Rituximab

According to the EULAR 2020 recommendations for the management of Sjogren’s syndrome, the first-line recommended therapy for cutaneous vasculitis involvement is glucocorticoids, while oral immunosuppressive agents or rituximab are the second-line recommended therapies.

Reference
Manuel Ramos-Casals et al. EULAR recommendations for the management of Sjogren’s syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3-18.
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Question 2

A 55-year-old female patient with rheumatoid arthritis is diagnosed with secondary Sjogren’s syndrome. She presents with painless, unilateral parotid gland swelling for over a month.

The following laboratory data is available:
• White cells count 2900 /µl (3000-9000 /µl) (neutrophils 62 %, lymphocytes 31 %)
• Haemoglobin 10.9 g/dl (12-16 g/dl)
• Platelet 25.6 × 104 /µl (15-35 ×104 /µl)
• Immunoglobulin G 1900 mg/dl (870-1700 mg/dl)
• Complement 3 84 mg/dl (80-140 mg/dl)
• Complement 4 8 mg/dl (11.0-34.0 mg/dl)
• Aspartate aminotransferase 22 U/L (7-38 U/L) • Alanine aminotransferase 18 U/L (4-44 IU/L)
• Serum cryoglobulin detected

Which of the following is NOT reported as lymphoma risk in this patient?
Question 2
- A. Persistent swelling of the salivary gland
- B. Cryoglobulinemia
- C. Lymphopenia
- D. Low complement 4
- E. Concomitant diagnosis of rheumatoid arthritis
Answer with a detailed explanation
E. Concomitant diagnosis of rheumatoid arthritis

The reported classical predictors of lymphoma in patients with Sjogren’s syndrome are persistent swelling of the salivary gland, adenopathy, purpura, cryoglobulinemia, lymphopenia, low complement 4, monoclonal component, and germinal centre-like structures within salivary gland/focus score. Concomitant diagnosis of rheumatoid arthritis is not reported as a lymphoma risk.

Reference Gaetane Nocturne, et al. Lymphomas complicating primary Sjogren’s syndrome: from autoimmunity to lymphoma. Rheumatology. 2021;60(8):3513-3521.
By Dr Masayuki Nishide, Assistant Professor, Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Japan
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Step 1 of 2

Question 1

A 38-year-old woman with primary Sjogren’s syndrome develops severe cutaneous vasculitis refractory to the initial treatment of glucocorticoids. What should be the next step in managing her condition?

Answer with a detailed explanation

Your Answer is Incorrect

Your Answer is Incorrect

Your Answer is Incorrect

Question 2

A 55-year-old female patient with rheumatoid arthritis is diagnosed with secondary Sjogren’s syndrome. She presents with painless, unilateral parotid gland swelling for over a month.

The following laboratory data is available:

Which of the following is NOT reported as lymphoma risk in this patient?

Answer with a detailed explanation

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