Thalidomide　therapy　for　granulomatous　interstitial　pneumonia　in　patients　with　chronic　granulomatous　disease

Abstract

Chronic　granulomatous　disease（CGD）is　a　primary　immunodeficiency　characterized　by　an　inability　of phagocytes　to　produce　reactive　oxygen　species， resulting　in　recurrent　life-threatening　infections．　Although　CGD patients　frequently　develop　inflammatory　granuloma　due　to　prolonged　production　of　inflammatory　cytokines，　the treatment　with　corticosteroid　and　immunosuppressive　drugs　are　limited　because　the　anti-inflammation　therapy for granuloma　formation　would　increase　susceptibility　to　infection　in　CGD．　Herein　we　described　two　CGD patients　who　suffered　from　granulomatous　interstitial　pneumonia (GIP) and　received　thalidomide　therapy　for three　months．　Their　serum　level　of　KL-6　was　high　and　CT　lung　images　showed　increased　reticulonodular opacities．　Pathological　findings　for　GIP　patient　reveal　inflammation　and　micro-granuloma　formation　of　interstitial lung　tissue，　which　are　similar　to　those　of　hypersensitivity　pneumonia．　Reticulo-nodular　opacities　on　CT　image were　disappeared　and　the　KL-6 1evels　were　reduced　after　three　months　of　thalidomide　therapy　in　CGD　patients with　GIP．Although　further　works　are　required，our　findings suggest　that　thalidomide　therapy　would　be therapeutic　effect　on　pulmonary　inflammatory　granuloma　in　the　CGD　patients．