Background: Common variable immunodeficiency(CVID) encompasses a heterogeneous group of antibody deficiency diseases defined by hypogammaglobulinemia, poor responses to vaccination, and recurrent infections. About a third of these patients show signs of autoimmunity or develop immune-mediated diseases. Among the oninfectivecomplications in CVID the lung may be involved in the course of Sarcoidosis- like syndromes or Granulomatous-lymphocytic interstitial disease (GLILD). Method: We recently observed a woman with history of recurrent infections of upper and lower airways, which progressed over the years from selective IgA deficiency to full-blown CVID with autoimmune haemolytic anemia, nodular lymphoid hyperplasia of gut and reactive lymph node hyperplasia. However, since 2005 she presented worsening of respiratory symptoms and lung infiltrates on chest X rays, which were diagnosed as Sarcoidosis and treated with courses of steroids, resulting in transient clinical improvement followed by multiple relapses. In 2012 we evaluated the patient, which underwent to bronchoscopy and lung biopsy, finally demonstrating the presence of Lymphocytic Interstitial Pneumonia and granulomatous lesions. Results: She has been treated with steroids and started intravenous immunoglobulin (Ig) substitution, then switched to Subcutaneous Ig replacement. After 6 months a clear improvement of symptoms and of infectious episodes was observed, nevertheless chest CT showed the presence of focal areas of ground-glass attenuation, particularly evident in the right middle lobe. Focal areas of consolidation with air bronchogram were evident in the right upper and middle lobe as well as in the left upper lobe. Moreover, some nodules of various sizes, most with irregular margins and some with associated thickening of the adjacent interlobular septa, were also found. By comparing the CT pattern in the scans obtained in 2004, 2011 and 2012 we found that the areas of ground glass attenuation were not modified whereas the number and dimensions of the nodules increased. Conclusion: Therapy was adjusted adding high-dose intravenous steroids and immunosuppresants. GLILD or Sarcoidosis-like syndromes or granulomatoid disorders could be one of the clinical presentations of CVID patients. Treatment with steroids and IgG replacement are the mainstay of treatment, being effective in most patients. However a close clinical and radiologic surveillance is required for this patients.
Granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency: a case report
FIRINU, DAVIDE;SABA, LUCA;MANCONI, PAOLO EMILIO;DEL GIACCO, STEFANO
2013-01-01
Abstract
Background: Common variable immunodeficiency(CVID) encompasses a heterogeneous group of antibody deficiency diseases defined by hypogammaglobulinemia, poor responses to vaccination, and recurrent infections. About a third of these patients show signs of autoimmunity or develop immune-mediated diseases. Among the oninfectivecomplications in CVID the lung may be involved in the course of Sarcoidosis- like syndromes or Granulomatous-lymphocytic interstitial disease (GLILD). Method: We recently observed a woman with history of recurrent infections of upper and lower airways, which progressed over the years from selective IgA deficiency to full-blown CVID with autoimmune haemolytic anemia, nodular lymphoid hyperplasia of gut and reactive lymph node hyperplasia. However, since 2005 she presented worsening of respiratory symptoms and lung infiltrates on chest X rays, which were diagnosed as Sarcoidosis and treated with courses of steroids, resulting in transient clinical improvement followed by multiple relapses. In 2012 we evaluated the patient, which underwent to bronchoscopy and lung biopsy, finally demonstrating the presence of Lymphocytic Interstitial Pneumonia and granulomatous lesions. Results: She has been treated with steroids and started intravenous immunoglobulin (Ig) substitution, then switched to Subcutaneous Ig replacement. After 6 months a clear improvement of symptoms and of infectious episodes was observed, nevertheless chest CT showed the presence of focal areas of ground-glass attenuation, particularly evident in the right middle lobe. Focal areas of consolidation with air bronchogram were evident in the right upper and middle lobe as well as in the left upper lobe. Moreover, some nodules of various sizes, most with irregular margins and some with associated thickening of the adjacent interlobular septa, were also found. By comparing the CT pattern in the scans obtained in 2004, 2011 and 2012 we found that the areas of ground glass attenuation were not modified whereas the number and dimensions of the nodules increased. Conclusion: Therapy was adjusted adding high-dose intravenous steroids and immunosuppresants. GLILD or Sarcoidosis-like syndromes or granulomatoid disorders could be one of the clinical presentations of CVID patients. Treatment with steroids and IgG replacement are the mainstay of treatment, being effective in most patients. However a close clinical and radiologic surveillance is required for this patients.
I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
