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Granulomatous–lymphocytic interstitial lung disease (GLILD) is a lung complication of common variable immunodeficiency disorders (CVID). It is seen in approximately 15% of patients with CVID. [1] It has been defined histologically as the presence of (non-caseating) granuloma and lymphoproliferation in the lung. [1]
A sarcoidosis-like lung disease called granulomatous–lymphocytic interstitial lung disease can be seen in patients with common variable immunodeficiency (CVID) and therefore serum antibody levels should be measured to exclude CVID. [citation needed]
Lymphocytic infiltration to tissues may cause enlargement of lymph nodes (lymphadenopathy), of the spleen (splenomegaly), and of the liver (hepatomegaly), as well as the formation of granulomas. In the lung, this is known as granulomatous–lymphocytic interstitial lung disease .
Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in 1972. [1] Lymphomatoid means lymphoma-like and granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymorphic lymphoid infiltrates and focal necrosis within it.
CHAI disease is a rare genetic disorder of the immune system that illustrates the role of CTLA-4 in cell signaling.CHAI stands for “Autoimmune lymphoproliferative syndrome due to CTLA4 haplo-insufficiency.” [1] [2] The disease is characterized by variable combination of enteropathy, hypogammaglobulinemia, recurrent respiratory infections, granulomatous lymphocytic interstitial lung disease ...
Alcoholic lung disease; Alpha-1 antitrypsin deficiency; ... Granulomatous–lymphocytic interstitial lung disease; Growth delay-hydrocephaly-lung hypoplasia syndrome; H.
The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas. [ 34 ] The full clinical picture was first presented by Friedrich Wegener (1907–1990), a German pathologist , in two reports in 1936 and 1939, leading to the eponymous name Wegener's granulomatosis or Wegener ...
The Epstein-Barr virus, which infects >90% of the world population, is also a common cause of these disorders, being responsible for a wide range of non-malignant, pre-malignant, and malignant Epstein-Barr virus-associated lymphoproliferative diseases. [5]