Related Experiment Video
Updated: Dec 12, 2025
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
20.7K
IgG4-related lung disease progressing to respiratory failure
Yusuke Kunimatsu1, Nozomi Tani1, Izumi Sato1
1Department of Respiratory Medicine Japanese Red Cross Kyoto Daini Hospital Kyoto Japan.
Respirology Case Reports
|August 14, 2020
Summary
Immunoglobulin G4 (IgG4)-related lung disease (IgG4-RLD) can cause rare respiratory failure. Prompt corticosteroid treatment effectively reversed respiratory failure in a 71-year-old patient.
Area of Science:
- Pulmonology
- Rheumatology
- Immunology
Background:
- Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fibroinflammatory disorder.
- IgG4-related lung disease (IgG4-RLD) comprises 13-14% of IgG4-RD cases.
- Respiratory failure is an uncommon manifestation of IgG4-RLD.
Observation:
- A 71-year-old male presented with respiratory failure after a 10-month observation period for interstitial lung disease.
- Diagnosis of IgG4-RD was confirmed by elevated serum IgG4, characteristic histopathology, and IgG4/IgG ratios.
- FDG-PET/CT revealed significant FDG uptake in lung fields, suggesting proliferative and reversible disease.
Findings:
- Systemic corticosteroid therapy rapidly improved the patient's respiratory failure.
- The patient achieved full recovery from respiratory failure.
- FDG accumulation indicated a proliferative and steroid-responsive state of IgG4-RLD.
Implications:
- This case highlights the potential for IgG4-RLD to cause severe respiratory failure.
- Early diagnosis and prompt corticosteroid treatment are crucial for favorable outcomes in IgG4-RLD.
- FDG-PET/CT can be a valuable tool in assessing disease activity and reversibility in IgG4-RLD.
Related Concept Videos
01:30Acute Respiratory Failure-III
595
Hypercapnic respiratory failure, also known as Type 2 or ventilatory respiratory failure, is a severe condition characterized by the body's inability to effectively remove carbon dioxide (CO2) from the bloodstream. It leads to an arterial CO2 pressure (PaCO2) exceeding 45 mmHg and a blood pH above 7.35. This situation indicates that the body's ventilatory demand, or the ventilation needed to maintain normal PaCO2 levels, surpasses its supply or the maximum gas flow achievable without...
595
01:23Acute Respiratory Failure-IV
408
Respiratory failure can manifest suddenly or gradually, characterized by a rapid decline in PaO2 and a rapid rise in PaCO2. This situation indicates a severe respiratory problem that may quickly become a life-threatening emergency. One of the early signs of hypoxemic Acute Respiratory Failure (ARF) is a change in mental status due to the brain's sensitivity to oxygen levels and changes in acid-base balance. Symptoms such as restlessness, confusion, and agitation suggest inadequate oxygen...
408
01:21Acute Respiratory Failure-I
633
Acute respiratory failure is a condition characterized by the inability of the lungs to perform their primary function: gas exchange. This failure leads to insufficient oxygen levels (hypoxemia) in the blood, elevated carbon dioxide levels (hypercapnia), or both, causing critical impairment in organ function.
Definition: It is defined by specific criteria based on blood gas measurements. Hypoxemia happens when the partial pressure of oxygen (PaO2) falls below 60 mmHg. At the same time,...
Definition: It is defined by specific criteria based on blood gas measurements. Hypoxemia happens when the partial pressure of oxygen (PaO2) falls below 60 mmHg. At the same time,...
633
01:29Acute Respiratory Failure-V
340
The treatment for acute respiratory failure varies based on factors like the underlying cause, overall health, and severity. A collaborative healthcare team is essential for early detection, often through arterial blood gas analysis. Identifying the cause is the primary goal, with treatment strategies adjusted for ventilation/perfusion (V/Q) mismatch, shunting, or diffusion impairment.
Ensure that patients are monitored continuously for their response to therapy, including changes in...
Ensure that patients are monitored continuously for their response to therapy, including changes in...
340
01:21Acute Respiratory Failure-II
858
Type I Respiratory Failure, or hypoxemic respiratory failure, occurs when the partial pressure of oxygen (PaO2) in arterial blood falls below 60 mmHg while breathing room air without a corresponding increase in arterial carbon dioxide levels (PaCO2). This condition highlights a significant impairment in the lungs' capacity to oxygenate the blood.
The underlying physiological abnormalities that contribute to hypoxemic respiratory failure include:
The underlying physiological abnormalities that contribute to hypoxemic respiratory failure include:
858
01:25Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.
3.3K
Understanding the variety of primary symptoms and systemic complications that characterize chronic obstructive pulmonary disease (COPD) is crucial for healthcare professionals.
Symptoms of COPD can be classified as primary or systemic. Primary symptoms relate to reduced airflow, while systemic or extrapulmonary symptoms relate to COPD's broader impact on the body.
Primary Symptoms of COPD:
Symptoms of COPD can be classified as primary or systemic. Primary symptoms relate to reduced airflow, while systemic or extrapulmonary symptoms relate to COPD's broader impact on the body.
Primary Symptoms of COPD:
3.3K