Sarcoidosis + honeycomb lung Flashcards
what is sarcoidosis
Systematic disease of unknown etiology characterized by the presence of non-caseating granulomas in many organs.
The sarcoidosis is seen commonly in females of which age
20-40 years
The sarcoidosis is associated with which antigen
HLA-A1
HLA-B8
list 4 immunological abnormalities associated with sarcoidosis
- Intra-alveolar and interstitial accumulation of CD4 + T cells resulting in CD4 :CD8 T cell ratio ranging from 5:1 to 15:1.
- Increased levels of IL-2 and IFN-g causing T-cell expansion and macrophage activation
respectively. - Polyclonal hypergammaglobulinemia.
- Anergy to skin antigens like purified protein derivative (PPD)Q.
Explain histologically appearance of sarcoidosis
Histologically, there is characteristically presence of non-caseating granuloma composed of aggregates of epithelioid cells and giant cells. There is also presence of Schaumann bodies (laminated concretions of calcium and protein) and asteroid bodies (stellate or star shaped inclusions in giant cells).
Explain the sarcoidosis in the lungs
Most common site of organ involvement
There is presence of non-caseating granuloma in the bronchial submucosa
Bronchoalveolar lavage shows CD4,CD9,T lymphocytes ration of>2.5 is seen
explain the sarcoidosis in lymph nodes
Involvement of hilar and mediastinal nodes is seen in almost all the cases
explain the sarcoidosis in the liver and spleen
splenomegaly may be seen with sparing of capsule. Scattered granulomas are seen more in portal triad as compared to globular paranchyma
explain the sarcoidosis in the bone marrow
favoured site of localization having tendency to involve phalangeal bones of hands and feet showing small areas of bone resorption, bony shaft widening and new bone formation
explain the sarcoidosis in the skin
lesion inclide erythema nodosum, subcutaneous nodules, erythematous plaque and lupus perniq
explain the sarcoidosis in eye, lacrimal and salivary glands
Unilateral or bilateral oculr involvement resulting in iritis, glaucoma or corneal opacity may occur.
Causes lacrimal gland inflammation (causing dry eyes) and salivary gland involvement (dry mouth)
Organizing diffuse alveolar damage is known as what?
Honeycomb lung
explain the honeycomb lung gross appearance
Honeycomb change, gross
Appearance of the irregular residual small dilated airspaces between bands of dense fibrous interstitial connective tissue.
The lung compliance is markedly diminished so that patients receiving mechanical ventilation require increasing positive end-expiratory pressure (PEEP), predisposing them to airway rupture and development of interstitial emphysema.