Makindo Medical Notes

The exact cause of Hamman-Rich Syndrome is unknown, but it is believed to be a severe and idiopathic form of lung injury. Unlike many other interstitial lung diseases (ILDs), it is not typically associated with environmental exposures, medications, or systemic diseases.

About

• First described in 1944.
• An acute and aggressive form of idiopathic pulmonary fibrosis (IPF).
• Also known as Acute Interstitial Pneumonia (AIP).

Aetiology

• Characterized by acute hypoxic respiratory failure with bilateral lung infiltrates.
• A rare and severe condition affecting previously healthy individuals.
• Unlike other forms of interstitial lung disease, it is not associated with cigarette smoking.
• It may represent a form of acute respiratory distress syndrome (ARDS) but with unknown etiology.

Clinical

• Rapid onset and progression from initial respiratory symptoms to severe respiratory distress and failure.
• Progressive dyspnoea, cyanosis, and hypoxia.
• Often presents with symptoms of a flu-like illness before rapid progression to acute respiratory failure.
• May require emergency care due to severe respiratory distress.

Investigations

• Spirometry: Shows a restrictive pattern.
• Reduced gas transfer: Indicating impaired oxygen exchange.
• CT Chest: Diffuse ground-glass opacities and consolidation, suggesting widespread inflammation and fluid accumulation.
• Echocardiogram: Helps exclude cardiac causes of respiratory failure, such as heart failure.
• Lung Biopsy: Required for definitive diagnosis, showing diffuse alveolar damage (DAD) and interstitial fibrosis, often indistinguishable from ARDS.

Management

• Supportive Care: Intensive care with mechanical ventilation is often necessary for patients with severe hypoxia.
• Ventilation: Intubation and ventilation are required for severe Type 1 respiratory failure (RF).
• Steroids: Generally poorly responsive to corticosteroid therapy, unlike some other forms of interstitial lung disease.
• Prognosis: The condition has a poor prognosis, with survival often measured in months due to rapid progression to fibrosis and respiratory failure.
• Palliative Care: In cases with refractory hypoxia and no response to treatment, palliative measures may be considered to manage symptoms and improve the quality of life.