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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

[Interstitial lymphoid pneumonia associated with common variable immunodeficiency].

Gonzalo Peralta1, Renzo Villagomez, Martin Bosio

  • 1Servicio de Neumonologia, Hospital Británico de Buenos Aires. gonzaperalta@gmail.com

Medicina
|November 8, 2011
PubMed
Summary
This summary is machine-generated.

Lymphoid interstitial pneumonia (LIP) is a rare lung disorder. This case highlights LIP in a patient with Sjogren syndrome, successfully treated with steroids and IVIG, alongside splenectomy for thrombocytopenia.

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Area of Science:

  • Pulmonology
  • Immunology
  • Pathology

Background:

  • Lymphoid interstitial pneumonia (LIP) is an uncommon interstitial lung disease characterized by non-neoplastic lymphoid tissue hyperplasia.
  • LIP is often associated with conditions like HIV, autoimmune disorders, and dysproteinemias, including hypogammaglobulinemia.

Observation:

  • A 66-year-old female with diabetes, Sjogren syndrome, and hypertension presented with cough and dyspnea.
  • Physical exam revealed bilateral crackles and splenomegaly. Lab results showed thrombocytopenia and hypogammaglobulinemia.
  • Thoracic CT scan demonstrated bilateral basal lung infiltrates and peribroncovascular nodules, confirmed as LIP via lung biopsy.

Findings:

  • The patient received oral methylprednisone and monthly intravenous immunoglobulin (IVIG), leading to significant clinical and radiological improvement.
  • Splenectomy was performed for persistent thrombocytopenia due to hypersplenism, resulting in normalized platelet counts.
  • No evidence of lymphoid clonal disorders or other associated conditions was found.

Implications:

  • This case underscores the importance of considering LIP in patients with autoimmune conditions and interstitial lung disease.
  • Combined treatment of steroids, IVIG, and splenectomy can be effective in managing LIP and its associated complications like thrombocytopenia.
  • Further research into the pathogenesis and optimal management of LIP, particularly in the context of autoimmune diseases, is warranted.