Pulmonary calcinosis associated with Alport syndrome

Area of Science:

• Nephrology
• Pulmonology
• Radiology

Background:

• Chronic renal failure (CRF) can lead to various complications.
• Alport syndrome is a genetic disorder affecting the kidneys, often leading to CRF.
• Metastatic pulmonary calcinosis is a rare complication of long-standing CRF.

Observation:

• A 48-year-old woman with a 29-year history of CRF secondary to Alport syndrome presented with diffuse micronodules on chest X-ray.
• Computed tomography revealed centrilobular, partially calcified micronodules, predominantly in upper lung regions and sparing subpleural spaces.
• Bone scan confirmed metastatic pulmonary calcinosis.

Findings:

• This is the first reported instance of pulmonary calcinosis occurring in the context of CRF specifically caused by Alport syndrome.
• The imaging findings suggest a slow, progressive development of metastatic calcification within the lung parenchyma over nearly three decades.
• The distribution pattern of calcifications provides insights into potential pathophysiological mechanisms in this specific patient population.

Implications:

• This case highlights the importance of considering rare pulmonary manifestations in long-standing CRF, particularly in patients with genetic kidney diseases like Alport syndrome.
• Further research may elucidate the specific mechanisms linking Alport syndrome, CRF, and pulmonary calcinosis.
• Radiologists and nephrologists should be aware of this potential complication for accurate diagnosis and management.