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RS Pathology
Respiratory System Pathology - Restrictive Lung diseases
| Question | Answer |
|---|---|
| What are restrictive lung diseases? | A group of disorders characterized by bilateral, patchy, chronic involvement of the lung connective tissue, mainly the interstitium in the alveolar walls |
| The interstitium is composed of | Basement membrane of the endothelial and epithelial cells , collagen and fibers and fibroblasts |
| The hallmark feature of these disorders is | Reduced compliance (i.e., more pressure is required to expand the lungs because they are stiff), which in turn necessitates increased effort of breathing (dyspnea) |
| Chest radiographs show | diffuse infiltration by small nodules, irregular lines, or "ground-glass" shadow |
| When restrictive lung diseases progress, patients can develop: | Respiratory failure Pulmonary hypertension Cor pulmonale |
| Advanced forms of these diseases may be difficult to differentiate because they result in: | Scarring and gross destruction of the lung, referred to as "honeycomb" lung |
| Restrictive lung diseases are divided into two groups: | 1) Granulomatous diseases 2) Fibrosing diseases |
| Sarcoidosis is an example of a: | Granulomatous disease |
| Sarcoidosis is a multisystem disease of unknown etiology characterized by: | noncaseating granulomas in many tissues and organs |
| The histologic diagnosis of sarcoidosis is one of | Exclusion |
| Epidemiology of sarcoidosis: | There is a consistent predilection for adults younger than 40 African Americans Higher prevalence among nonsmokers |
| Etiology of sarcoidosis: | A disease of disordered immune regulation in genetically predisposed persons exposed to certain environmental agents |
| Immunologic abnormalities in sarcoidosis suggest the development of a cell-mediated response to an unidentified antigen. This process is driven by: | CD4+ helper T cells |
| Immunologic abnormalities in sarcoidosis include: | 1) Intra-alveolar and interstitial accumulation of CD4+ TH1 cells 2) Increases in T cell-derived TH1 cytokines such as IL-2 and IFN-γ, resulting in T cell expansion and macrophage activation, respectively 3) Anergy to common skin test antigens like PPD |
| The role of genetic factors in sarcoidosis is suggested by: | 1) Familial clustering of cases 2) Association with certain human leukocyte antigens (HLA) ( class I HLA-A1 and HLA-B8) |
| After lung transplantation, sarcoidosis recurs in the new lungs in what percentage of patients? | 75% |
| Morphology of sarcoidosis: | Noncaseating epithelioid granuloma Schaumann bodies, laminated concretions composed of calcium and proteins Asteroid bodies, stellate inclusions enclosed within giant cells |
| Involved organs in sarcoidosis: | The lungs in 90% Intrathoracic hilar and paratracheal lymph nodes enlarged in 75% to 90% 1/3 present with lymphadenopathy Skin lesions are encountered in 25% Eye and lacrimal glands 1/5 Parotitis Spleen, liver and Bone marrow |
| In 5% to 15% of patients, the granulomas eventually are replaced by | diffuse interstitial fibrosis, resulting in a so-called honeycomb lung |
| The hallmark of acute sarcoidosis is: | Erythema nodosum |
| Erythema nodosum is characterized by: | Raised, red, tender nodules on the anterior aspects of the legs |
| Subcutaneous nodules are | Painless, discrete Reveal abundant noncaseating granulomas |
| Ocular involvement takes the form | Iritis Iridocyclitis Unilateral or bilateral Total loss of vision may develop Inflammation of lacrimal glands Suppression of lacrimation (Sicca syndrome) |
| In parotitis, there is: | Unilateral or bilateral parotitis with painful enlargement of the parotid glands Some patients develop xerostomia (dry mouth). Combined uveoparotid involvement is designated Mikulicz syndrome. |
| Hypercalcemia in sarcoidosis is caused by: | Increased calcium absorption secondary to production of active vitamin D by the mononuclear phagocytes in the granulomas |
| Clinical features of sarcoidosis: | In many affected persons the disease is a. Asymptomatic b. Discovered bilateral hilar adenopathy c. Incidental finding at autopsy Peripheral lymphadenopathy Cutaneous lesions Eye involvement Splenomegaly Hepatomegaly Resp. symptoms |
| The presence of noncaseating granulomas is suggestive of sarcoidosis, but | Other identifiable causes of granulomatous inflammation must be excluded |
| Clinical course of sarcoidosis: | Unpredictable course characterized by either progressive chronicity or periods of activity interspersed with remissions |
| Recovery from Sarcoidosis: | Remissions spontaneous or initiated by steroid therapy- often are permanent 65% to 70% - recover with minimal or no manifestations 20% - permanent lung dysfunction or visual impairment 10% to 15% - progressive pulmonary fibrosis and cor pulmonale |
Created by:
Ulaisl
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