Infections and auto-immune diseases I Flashcards
In case of immune dysregulation, there can be… (3)
- Immunodeficiency
- Autoinflammation
- Autoimmune disease
What kind of disease is sarcoidosis?
Granulomatous disease
Sarcoidosis: What is the result of the hyper activation of the immune system?
Collections of dysregulated overactive T cells, macrophages and epithelioid cells
What is are examples of factors created by the granuloma’s in sarcoidosis? (2)
- Soluble IL-2-R
- Angiotensin converting enzyme (ACE)
True or false: soluble IL-2 receptor is marker for active granuloma formation
True
What is the current treatment for sarcoidosis? (2)
- Corticosteroids
- DMARDS, biologicals
What is a clinical hallmark of PID?
Recurrent and severe infections
What are characteristics of CVID? (4)
- Recurrent respiratory- and GI tract infections
- Autoimmunity
- Increased risk of malignancy (lymphoma)
- Low IgG, IgA and/or IgM
Where does XLA interfere with proper B cell development?
BTK inhibition -> between pro-B cell and Pre-B cell
What are the three deficiencies that occur in CVID?
- IgA deficiency
- Hyper IgM syndrome/other CSR defects
- IgG subclass deficiency
What is PAD?
Primary antibody deficiency
There is no increased risk for infectious complications in sarcoidosis, except cryptococcosis. Why cryptococcosis?
Th1 cytokine production protects against infection with cryptococcosis -> Alveolar macrophages produce IL-10 downregulating Th1 response
What is the treatment of patients with primary antibody deficiency complicated by auto inflammation? (3)
- Immunoglobulin replacement therapy
- Antibiotics
- Immunosuppressants
Which two types of non-infectious complications occur in PID?
- Hematological malignancies
- Autoimmune complications
Where are granuloma’s in CVID most prevalent?
Lungs
