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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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Glomerulonephritis associated with systemic sclerosis: a case report.

Sepehr Nayebirad¹, Alireza Ramandi², Fatemeh Nili³

¹Students' Scientific Research Center (SSRC), Tehran University of Medical Sciences, Tehran, Iran.

Journal of Medical Case Reports

|February 9, 2023

Summary

This case study highlights a rare instance of systemic sclerosis overlapping with antineutrophil cytoplasmic antibody-associated vasculitis in a patient presenting with nasal perforation and glomerulonephritis. Early recognition of overlapping conditions is crucial for effective management.

Keywords:

ANCA Glomerulonephritis Systemic sclerosis Vasculitis

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Area of Science:

Rheumatology
Nephrology
Autoimmune Diseases

Background:

Systemic sclerosis (SSc) is a complex autoimmune disease affecting multiple organs, occasionally overlapping with other rheumatologic conditions.
Co-occurrence of SSc with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is exceptionally rare, presenting diagnostic challenges.

Observation:

A 39-year-old female with a 6-year history of SSc presented with rising creatinine and nasal perforation.
She had a 5-year history suggestive of AAV, including purpura and hemoptysis, but declined biopsy.
Renal biopsy revealed crescentic glomerulonephritis with immune deposits, indicating active kidney inflammation.

Findings:

The patient's renal biopsy demonstrated significant glomerular damage, including cellular crescents and Bowman capsule rupture.
Immunofluorescence microscopy confirmed granular immune deposits in the glomeruli and tubular basement membranes.
This histological pattern, coupled with clinical presentation, confirmed comorbid SSc and AAV.

Implications:

This case underscores the importance of considering overlapping autoimmune conditions, even with rare presentations.
Uncommon manifestations like nasal perforation in SSc may signal underlying vasculitis.
Renal involvement in SSc, though infrequent, necessitates vigilance for glomerulonephritis and potential comorbid AAV or systemic lupus erythematosus.