T-cell-mediated immune defect and dysregulation (with B-cell overactivity) due to unknown etiology (EBV has been found in lymphatic tissue of AILD patients)
Disease presents similarly in HIV patients
Pathology
Pathology may resemble peripheral T-cell, Lennert’s, lymphoma or giant lymph node hyperplasia (Castleman’s disease)
Proliferation or arborization of blood vessels within enlarged lymph nodes or lymphatic tissue
Effacement of normal nodal archi-tecture by polymorphous inflamma-tory cell infiltrate
Depletion of normal lymphocytes and germinal centers
Amorphous eosinophilic material in intracellular spaces
Lack of Reed-Sternberg cells (excludes Hodgkin’s disease)
Diagnosis
CXR/Chest CT Patterns
Diffuse interstitial infiltrates
Pleural effusion/pleural thickening
Massive hilar/mediastinal adenopathy
CBC
Aplastic anemia: may occur
Autoimmune Hemolytic Anemia (40% of cases)
Leukocytosis (with lymphocytopenia/eosinophilia)
Thrombocytopenia (may be autoimmune)
Pleural Fluid: exudate
Other Autoantibodies: may occur
Paraproteinemia
Polyclonal hyperglobulinemia (70% of cases)
Hypoglobulinemia: may occur
Monoclonal hyperglobulinemia (occurs late in course)
Skin/Liver/Bone Marrow/Lung Biopsy: usually necessary for diagnosis
Clinical Presentation
Pleural Effusion (see [[Pleural Effusion-Exudate]])
Constitutional: fever, weight loss, fatigue
Skin: rash, pruritus
Lymphadenopathy (always present)
Hepatosplenomegaly:
Lymphoma (usually intermediate or high-grade types): develops in 5-20% of cases
Treatment
Benign course (33% of cases): mild disease or spontaneous remission
Progressive course (67% of cases): poor prognosis despite treatment
Danazol: reported efficacy for hemolysis/ thrombocytopenia in some cases
