Pulmonary involvement in angioimmunoblastic lymphadenopathy following autoimmune disease

A 44-year-old woman presented with a systemic illness characterized by a rash, polyarthritis and hypothyroidism. She later developed angioimmunoblastic lymphadenopathy (AIL) which became progressively resistant to chemotherapy. Pulmonary features were prominent throughout her illness and included pleural effusions, hilar adenopathy and parenchymal infiltrates. Lung histology showed a transition from nonspecific chronic inflammation with fibrosis to a polymorphic cellular infiltrate typical of AIL. Immunological features included a low C3 level, hypogammaglobulinaemia and depressed cell-mediated immunity.