Pulmonary-renal syndrome: diagnostic challenge

Area of Science:

• Nephrology
• Pulmonology
• Rheumatology

Background:

• Vasculitis poses significant diagnostic challenges, often presenting with non-specific symptoms.
• Early recognition and intervention are crucial for managing systemic autoimmune diseases.

Observation:

• A 71-year-old woman presented with asthenia, vomiting, cough, progressive renal failure, and anemia.
• Physical examination revealed pulmonary crackles; lab tests showed anemia, elevated creatinine, hyperkalemia, positive antineutrophil cytoplasm antibody (ANCA), and abnormal urinalysis.
• Thoracic CT scan indicated diffuse alveolar hemorrhage, suggesting pulmonary-renal syndrome.

Findings:

• The patient received induction therapy for presumed pulmonary-renal syndrome.
• Despite initial treatment and discharge, she required hemodialysis due to limited renal function recovery.
• While ANCA titers became undetectable, indicating disease control, the patient experienced only minor renal function improvement, underscoring treatment complexities.

Implications:

• Early aggressive induction therapy in vasculitis-associated pulmonary-renal syndrome can significantly improve patient outcomes.
• Maintaining long-term disease remission in vasculitis is challenging, with a high risk of relapse.
• This case emphasizes the need for vigilant monitoring and potentially adjusted long-term management strategies for vasculitis patients.