M257 flavonoids improve pulmonary assessments in granulomatous-lymphocytic interstitial lung disease with common variable immune deficiency
Granulomatous-lymphocytic interstitial lung disease (GLILD) is a restrictive lung disease, defined as pulmonary tissue containing both granulomatous and lymphoproliferative histopathologic patterns. GLILD is characteristically associated with common variable immune deficiency (CVID), and patients have poorer outcomes. Currently, there is no established standard of care for the treatment of GLILD in CVID.
We report a case of CIEP in a child with no known atopy or asthma.
This study assessed omalizumab outcomes in real-world patients with allergic asthma stratified by pretreatment biomarker levels. METHODS: Patients with allergic asthma aged ≥12 years initiated on omalizumab with ≥12 months of data after index were identified in the Allergy Partners electronic medical records (2007-2018). Patients with ≥1 diagnosis of chronic obstructive pulmonary disease in combination with ≥10 pack-years of smoking, cystic fibrosis, Alpha-1 antitrypsin deficiency, bronchiectasis, interstitial lung disease, and sarcoidosis in the 12 months before or after index were excluded. Patients were...
Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder caused by defective fas-mediated apoptosis. Patients often present in childhood with lymphoproliferation, splenomegaly and multilineage cytopenias (Price et al. Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations. Blood 2014). Though mutations in the FAS gene account for the majority of cases, an estimated 20% of patients who have no defined genetic cause are classified as ALPS-U (Shah et al. Autoimmune lymphoproliferative syndrome: an update and review of the literature. Current allergy and asthma reports 20...
COPA syndrome is a newly discovered primary immunodeficiency syndrome which consists of interstitial lung disease, pulmonary hemorrhage, inflammatory arthritis and renal disease. The majority of patients present with symptoms before the age of 5. Emerging case reports defining presentations of this syndrome have been important in further characterizing this disease.
We report a case of an adult female who presented initially with the clinical manifestations of granulomatous-lymphocytic interstitial lung disease (GILID) prior to CVID.
We describe the second reported case of GLILD in a patient with 22q11.2 deletion syndrome (22q11.2DS) and review the recent literature surrounding GLILD.Recent FindingsGLILD is characterized by granulomata and lymphoproliferation. Consensus statements and retrospective and case-control studies have better elucidated the clinicopathological and radiographic manifestations of GLILD, allowing for its differentiation from similar conditions like sarcoidosis. Gaps of knowledge remain, however, particularly regarding optimal management strategies. Combination therapies targeting T and B cell populations have recently shown favor...
While CVID is the most common form of clinically significant primary immunodeficiency, due to its heterogeneous features and complications, there continues to be a 5 year diagnostic delay. In patients with CVID, there is a 2-8% lifetime risk of lymphoma and about 10% develop granulomatous disease. Longer diagnostic delays, presence of malignancy, and granulomatous/lymphocytic interstitial lung disease (GLILD) are associated with increased risk of death. This 59 year old female, misdiagnosed with sarcoidosis, was found to have CVID-related GLILD and lymphoma.
The patient is a 22-year-old man with a history of common variable immunodeficiency (CVID) associated with a heterozygous mutation in TNFRSF13B (TACI), granulomatous lymphocytic interstitial lung disease (GLILD), massive splenomegaly, immune thrombocytopenia, and autoimmune hemolytic anemia. He presented with a 5-day history of fever with temperatures up to 38.9 °C, malaise, sinus congestion, intermittent headaches, and loose stools. He had no cough, dyspnea, vomiting, abdominal pain, joint pain, or rash.
We read with great interest the article by Hartono et al1 that was published in the Annals of Allergy, Asthma&Immunology in May 2017. They intended to determine a set of clinical and/or laboratory parameters associated with granulomatous lymphocytic interstitial lung disease (GLILD). Based on univariable analysis, they stated that splenomegaly was independently associated with GLILD (odds ratio [OR] 17.3, 95% confidence interval [CI] 3.9 –74.5). Although the study was useful and very interesting, some methodologic issues need to be considered.
PMID: 28890025 [PubMed - in process]