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RS Pathology

Respiratory System Pathology - Restrictive Lung diseases

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