Advancing IgG4-RD Care Through Early Diagnosis, Evidence-Based Treatment, and Multidisciplinary Care : Pre-test

1.

How many people with IgG4-related disease (IgG4-RD) do you provide care for in a typical month?

A. 1-4
B. 5-10
C. 11-15
D. 16-20
E. >20
F. Not applicable

2.

For those who practice in academic or community settings, please indicate your practice setting:

A. Academic
B. Community
C. Not applicable

3.

Which of the following statements regarding the diagnostic utility of serum IgG4 levels in IgG4-RD is most accurate according to current diagnostic consensus guidelines?

A. Normal serum IgG4 levels do not exclude IgG4-RD and elevated concentrations should be interpreted alongside clinical, radiologic, and histopathologic findings
B. A serum IgG4 level >135 mg/dL provides sufficient specificity to establish IgG4-RD when compatible organ involvement is present
C. Serial serum IgG4 measurements provide a reliable surrogate for disease activity and can guide treatment escalation or glucocorticoid tapering independently of clinical and imaging findings
D. Markedly elevated serum IgG4 concentrations reliably distinguish IgG4-RD from malignancy and other immune-mediated disorders, whereas normal concentrations make the diagnosis unlikely

4.

A 52-year-old woman with biopsy-proven IgG4-related dacryoadenitis and sialadenitis (Mikulicz disease) has symptomatic orbital and salivary gland enlargement causing functional discomfort, without end-organ failure.

What is the standard first-line medical therapy recommended by international consensus guidelines for induction of remission in symptomatic, active IgG4-RD?

A. Begin oral prednisone at 0.2 mg/kg/day with a prolonged taper, reserving higher doses for patients who develop organ dysfunction
B. Initiate rituximab monotherapy as the preferred first-line induction strategy, reserving glucocorticoids for inadequate response or relapse
C. Combine high-dose intravenous glucocorticoids with cyclophosphamide to achieve rapid remission and minimize subsequent relapse risk
D. Initiate oral prednisone at approximately 0.6 mg/kg/day, maintain the induction dose for 2-4 weeks, and then taper gradually according to clinical response

5.

A 64-year-old man with multisystem IgG4-RD involving the pancreas, kidneys, and retroperitoneum achieves remission following glucocorticoid induction. During tapering, he experiences recurrent abdominal discomfort and worsening renal function. His cumulative glucocorticoid exposure has also contributed to diabetes and osteoporosis.

Which management strategy is most appropriate to reduce further organ damage and support long-term disease control?

A. Reinstitute glucocorticoids and continue long-term maintenance at the lowest effective dose, with monitoring led primarily by the treating specialist
B. Coordinate care among relevant organ specialists, reassess disease activity and organ damage, and consider a glucocorticoid-sparing strategy based on relapse risk, treatment history, and patient-specific factors
C. Discontinue glucocorticoids and monitor serum IgG4 concentrations every 3 months, initiating treatment only if levels rise substantially
D. Initiate a biologic agent based on the elevated serum IgG4 concentration alone, with subsequent follow-up determined by biochemical response

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Advancing IgG4-RD Care Through Early Diagnosis, Evidence-Based Treatment, and Multidisciplinary Care

Yasmin G. Hernandez-Barco, MD

Guy Katz, MD, MS

Arezou Khosroshahi, MD

Jai Radhakrishnan, MD, MS

Chuck Vega, MD, FAAFP

Pre Assessment

Assess your current knowledge and clinical approach before beginning your text module.

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