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Primary Sjogren's Syndrome Associated with Lung Adenocarcinoma: Probing the Potential Common Pathogenic Mechanisms and Experimental Verification

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Mixed connective tissue disorder and Castleman's disease.

Anugrah Chrispal¹, Zoya Vasuki, Elsa Mary Thomas

¹Department of Medicine, Christian Medical College, Vellore.

The Journal of the Association of Physicians of India

|December 31, 2010

Summary

A rare case of Castleman's disease (a lymphoproliferative disorder) was diagnosed in a teenage girl with mixed connective tissue disorder. Immunosuppression may treat both conditions.

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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis

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Last Updated: Jun 5, 2026

Primary Sjogren's Syndrome Associated with Lung Adenocarcinoma: Probing the Potential Common Pathogenic Mechanisms and Experimental Verification

Primary Sjogren's Syndrome Associated with Lung Adenocarcinoma: Probing the Potential Common Pathogenic Mechanisms and Experimental Verification

Published on: September 20, 2024

An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis

Published on: February 8, 2019

Area of Science:

Immunology
Oncology
Nephrology

Background:

Mixed Connective Tissue Disorder (MCTD) is an autoimmune condition with overlapping features of other connective tissue diseases.
Castleman's disease is a rare lymphoproliferative disorder with diverse clinical presentations.
The co-occurrence of MCTD and Castleman's disease is exceptionally rare, posing diagnostic and therapeutic challenges.

Observation:

A 16-year-old female presented with a 6-month history of polyarthritis, Raynaud's phenomenon, malar rash, oral ulcers, photosensitivity, and alopecia.
Diagnostic workup revealed MCTD with mesangio-proliferative glomerulonephritis.
Imaging identified a hilar mass, later diagnosed as hyaline vascular type Castleman's disease via CT-guided biopsy.

Findings:

The patient exhibited a rare association between MCTD and Castleman's disease.
The clinical manifestations were varied, highlighting the complexity of this dual diagnosis.
The underlying etiopathogenesis linking autoimmune disease and Castleman's disease remains unclear.

Implications:

Understanding this rare association is crucial for effective patient management.
Immunosuppressive therapy may potentially address both the autoimmune manifestations of MCTD and the tumor regression in Castleman's disease.
Further research into the etio-pathogenesis is warranted to optimize treatment strategies for this rare comorbidity.