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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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Scleroderma and IgG4-related disease.

Deepti M Reddi¹, Diana M Cardona, James L Burchette

¹Department of Pathology, Duke University Medical Center, Durham, NC, USA.

The American Journal of Dermatopathology

|April 9, 2013

Summary

Scleroderma, a fibrotic condition, shows minimal elevation of IgG4-positive cells, unlike IgG4-related disease. This suggests scleroderma is distinct from IgG4-related disease spectrum, impacting autoimmune and fibrotic disorder research.

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Granulocyte-dependent Autoantibody-induced Skin Blistering

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Area of Science:

Immunology
Rheumatology
Pathology

Background:

Immunoglobulin G4 (IgG4)-related disease is a systemic condition characterized by lymphoplasmacytic infiltrates, tissue fibrosis, and elevated serum IgG4 levels.
Scleroderma is a fibrotic disorder that shares some histopathological features with IgG4-related disease, including fibrosis and lymphoplasmacytic infiltrates.
The presence and significance of IgG4-positive plasma cells in scleroderma have not been well-established.

Purpose of the Study:

To investigate and characterize the presence of IgG4-positive plasma cells in scleroderma and related fibrotic disorders.
To determine if scleroderma fits within the spectrum of IgG4-related disease based on IgG4-positive cell infiltration.

Main Methods:

A retrospective review of 34 cases of scleroderma and related conditions (CREST syndrome, progressive systemic sclerosis, morphea) was conducted.
IgG4-positive and IgG-positive plasma cells were quantified per 10 high-power fields (HPF).
An IgG4:IgG ratio was calculated, with a cutoff ratio of 0.3 used to define significant elevation.

Main Results:

Only 1 out of 34 scleroderma cases exhibited a significant elevation in the IgG4:IgG ratio (≥0.3).
Three cases showed an elevated IgG4:IgG ratio (>0), with a median ratio of 0.22.
Similar low levels of IgG4-positive cells were observed in other sclerosing disorders examined.

Conclusions:

Scleroderma and related fibrotic conditions do not typically display the elevated IgG4-positive plasma cell infiltration characteristic of IgG4-related disease.
The findings suggest that scleroderma is a distinct entity and likely not part of the IgG4-related disease spectrum.
This research clarifies the histopathological differences between scleroderma and IgG4-related disease.