Intravenous Immunoglobulin Monotherapy for Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency
نویسندگان
چکیده
Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease. Interestingly, the lung lesions of this case resolved immediately after the initiation of intravenous immunoglobulin monotherapy.
منابع مشابه
Common variable immunodeficiency
Common variable immunodeficiency (CVID) is a common primary immunodeficiency characterized by a failure in B-cell differentiation with defective immunoglobulin production. Affected patients are uniquely susceptible to recurrent infection with encapsulated organisms and have an increased propensity for the development of inflammatory and autoimmune manifestations. The diagnosis of CVID is common...
متن کاملBritish Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders.
A proportion of people living with common variable immunodeficiency disorders develop granulomatous-lymphocytic interstitial lung disease (GLILD). We aimed to develop a consensus statement on the definition, diagnosis, and management of GLILD. All UK specialist centers were contacted and relevant physicians were invited to take part in a 3-round online Delphi process. Responses were graded as S...
متن کاملLymphocytic interstitial pneumonia associated with common variable immunodeficiency resolved with intravenous immunoglobulins.
Lymphocytic interstitial pneumonia (LIP) is a rare form of interstitial lung disease. A few case reports have described an association with common variable immunodeficiency (CVID). Corticosteroids are usually used to treat symptomatic patients but their efficacy has never been studied in a controlled trial. We describe a patient with LIP and CVID who was treated monthly with intravenous immunog...
متن کاملA case of granulomatous lung disease in a patient with Good's syndrome
Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatenin...
متن کاملعوارض ناشی از تزریق ایمونوگلوبولین وریدی در بیماران با نقص ایمنی اولیه
Background and Purpose: Intravenous immunoglobulin is used for primary immunodeficiency disorders. There have been some reports that intravenous immunoglobulin causes side effects. The aim of this study was to investigate intravenous immunoglobulin side effects in immunodeficiency patients. Materials and Methods: The study utilized the data of 29 primary immunodeficiency patients that were...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 56 شماره
صفحات -
تاریخ انتشار 2017