Epidemiology
- Almost all patients are adults
- Associated with: Sjogren’s, HIV
Physiology
- 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
- Steroids/Cytotoxics: have been used
- CSA/Interferon-alpha: have been used
Prognosis
- Median survival: 1.5 years
- Most deaths occur due to infection
References
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