What is the mortality rate of Acute Intermittent Porphyria (AIP)?

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Last updated: May 14, 2025View editorial policy

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From the Guidelines

The mortality rate for Acute Interstitial Pneumonia (AIP) is approximately 60% with the majority of patients dying within 6 months of presentation. This high mortality rate is due to the rapid progression of the disease, which presents with symptoms such as fever, cough, and shortness of breath, and is characterized by diffuse, bilateral, airspace opacification on chest radiograph and CT scans showing bilateral, patchy, symmetric areas of ground glass attenuation 1. The diagnosis of AIP requires the presence of a clinical syndrome of idiopathic Acute Respiratory Distress Syndrome (ARDS) and pathological confirmation of organizing diffuse alveolar damage (DAD). Some key features of AIP include:

  • Moderate to severe hypoxemia and development of respiratory failure
  • Lung biopsies showing histologic features identical to those of the exudative, proliferative, and/or fibrotic phases of DAD
  • The exudative phase showing edema, hyaline membranes, and interstitial acute inflammation
  • Loose organizing fibrosis mostly seen within alveolar septa, but it may also be seen within airspaces The main treatment for AIP is supportive care, and the high mortality rate highlights the importance of early recognition and intervention 1.

From the Research

Acute Invasive Fungal Sinusitis (AIFS) Mortality

  • AIFS is an aggressive disease that requires prompt diagnosis and multidisciplinary treatment given its rapid progression 2.
  • The mortality rate for AIFS is high, but the exact rate is not specified in the provided studies.
  • A multidisciplinary approach to management, including surgery, antifungal therapy, and correcting sources of immunosuppression, is recommended for optimal outcomes 2.
  • Higher quality studies are needed to better define the roles of each modality and determine diagnosis and treatment algorithms for AIFS 2.

Fluid Management and Mortality

  • Fluid overload has been shown to be associated with increased risk for mortality in sepsis patients 3.
  • A conservative or deresuscitative fluid strategy may result in increased ventilator-free days and reduced length of ICU stay compared to a liberal strategy or standard care 4.
  • However, the effect of fluid strategies on mortality remains uncertain, and large randomized trials are needed to determine optimal fluid strategies in critical illness 4.
  • The relationship between fluid administration or balance and clinically important patient outcomes in critical illness, including mortality, is still being studied and debated 5.

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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