Chronic Granulomatous Diseases

Question 1

Sarcoidosis

Answer 1

Systemic disease
unknown etiology
noncaseating naked granulomas many in tissues and organs

Question 2

Most common organ involved in sarcoidosis

Answer 2

the lung - Bilateral hilar lymphadenopathy and parenchymal involvement (90% of cases)

Question 3

Sarcoidosis incidence

Answer 3

Adults under 40 years
Male
black people

Question 4

Sarcoidosis pathogenesis

Answer 4

Disordered immune regulation and genetic predisposition with the unknown etiology

Question 5

Disordered immunity in sarcoidosis

Answer 5

Type 4 hypersensitivity with unidentified antigen
In granuloma:
Elevated T4 lymphocytes

elevated T cell dérivâted cytokines like IL-2 and INF-y

elevated other cytokines like TNF

Activated alveolar macrophages with class to HLA and APC activity

Question 6

Genetic susceptibility in sarcoidosis

Answer 6

Familial and racial clustering

Certain HLA genotypes associated with disease

Question 7

Sarcoidosis morphology

Answer 7

Non caseating/ necrotizing granuloma

Laminated concretions of calcium and proteins (schaumann bodies)

Stellate inclusion bodies in cytoplasm of giant cells (Asteroid bodies)

Question 8

Sarcoidosis presentation

Answer 8

Granuloma in:
lung 100% - become nodule in late disease which heal by fibrosis

lymph nodes 100%- hilar and mediastinal nodes, enlarged, calcification

spleen 75% - small nodules sometimes

skin and mucous membranes- painless subcutaneous nodules , red plaques

eyes 25%- iritis, corneal opacifies, glaucoma , blindness

Others : liver bone marrow salivary gland’s muscle

Question 9

Sarcoidosis Symptoms

Answer 9

Interstitial lung disease : 
dyspnea 
Cough  
Chest pain  
hemoptysis
 fatigue 
weight loss 
Fever  
anorexia 
night sweats

Question 10

Treatment of sarcoidosis

Answer 10

Steroids

Question 11

Prognosis of sarcoidosis

Answer 11

65-70 % recover completely

20% permanent loss of lung and vision

10-15% die ( lung fibrosis, cor pulmonary, cardiac or CNS damage)

Question 12

Tuberculosis

Answer 12

Chronic pulmonary and systemic disease caused by Mycoplasma tuberculosis and sometimes mycoplasma bovis

Question 13

Reservoir in hosts of tuberculosis

Answer 13

Humans with active tuberculosis tuberculous dairy cows

Question 14

Transmission of tuberculosis

Answer 14

Inhalation of sputum containing organism

drinking unpasteurized milk

Question 15

Cause of tuberculosis spread worldwide

Answer 15

HIV AIDS
malnutrition and parasitic infection in poor countries
Multi drug resistance of TB

Question 16

People at risk of tuberculosis

Answer 16

Elderly 
urbanpoor 
AIDS patients 
diabetes mellitus
 Hodgkin’s lymphPhoma 
chronic lung disease 
silicosis 
chronic renal failure 
Malnutrition 
alcoholism 
immunosuppression

Question 17

Pathogenesis of tuberculosis

Answer 17

1st Exposure of immunocompetence individual ( T cell mediated response contain bacteria and tissue damage, phagocytosis of organism but no intracellular hkilling, replication in macrophages, )

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Question 18

Two Pathophysiological types of tuberculosis

Answer 18

Primary tuberculosis - Previously unexposed and unsensitized individuals

secondary tuberculosis - Previously exposed and therefore immune individuals

Question 19

Morphology of tuberculosis In primary tuberculosis

Answer 19

Pulmonary lesions
Ghon focus & ghon complex &
Ranke complex

Epithelioid cells (activated macrophages with indistinct cell boundaries) l

Caseous necrosis