Conclusion
This case demonstrates an increasingly important presentation of a somewhat rare disease. This disease can affect any organ and is important to consider in any differential diagnosis of saddle nose deformity.
Alyxandra Soloway, MD, is an internal medicine resident in the Department of Medicine of Inspira Health Network in Vineland, N.J.
Stephen Soloway, MD, FACP, FACR, is chair of the Division of Rheumatology, Department of Medicine, Inspira Health Network in Vineland, N.J.
Classification Criteria for IgG4-Related Disease
The ACR and EULAR published classification criteria for IgG4-related disease (IgG4-RD) in the January 2020 issue of Arthritis & Rheumatology. The criteria were developed primarily to identify patients for inclusion in clinical trials and other studies, and are not intended for diagnostic use in clinical practice.
The three-step classification process is:
- First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD.
- Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification.
- Third, eight weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments and pathology interpretations, are applied.
References
- Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb;366(6):539–351.
- Cheuk W, Chan JK. IgG4-related sclerosing disease: A critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010 Sep;17(5):303–332.
- Schreiber BE, Twigg S, Marais J, et al. Saddle-nose deformities in the rheumatology clinic. Ear Nose Throat J. 2014 Apr-May;93(4-5):E45–47.
- Khosroshahi A, Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 2011 Jan;23(1):57–66.
