I Restrictive Pulmonary Disorders a Due to decreased lung expansion b Etiological classification i Parenchymal lung disorders fibrotic interstitial lung disease atelectatic disorders ii Pleural space disorders between parietal viseral iii Chest wall disorders kyphoscoliosis ankylosing spondylitis flail chest disorders of obesity adding fat to chest wall increases pressure and decreases space to expand iv Neuromuscular disorders v Pulmonary infection inflammation pneumonia severe acute II respiratory syndrome pulmonary tuberculosis Incidence 5 100 000 Fibrotic Interstitial Lung Disease a Etiology unknown b Alveolar wall infiltration by cells fluid and connective tissue c d Acute allergies minutes subacute chronic e Can progress to irreversible fibrosis if untreated f Pathogenesis i Immune reaction initial injury to alveolar epithelium or capillary endothelium interstitial and alveolar wall thickening increased interstitial collagen bundles Inflammation early and reversible triggers cause inflammation and increased inflammatory cells injury increases membrane permeability and fluid debris movement into alveoli ii 1 Stretching capiilary pores 2 Mast cells neutrophils macrophages eaosiniphils iii Fibrosis fibroblastic proliferation and collagen deposition due to increased interstitial mesenchymal cells and fibroblasts alveolar walls thicken with increased fibrous tissue cracking at alveoli and destruction will eventually kill the patient iv Destruction end stage disease loss of alveolar walls g Clinical manifestations i Progressive dyspnea with exercise and desaturation ii Rapid shallow breathing iii Irritating dry cough iv Nail bed clubbing bibasilar both bases of lungs end expiratory crackles v Late cyanosis vi Anorexia weight loss no energy vii h Diagnosis Inability to increase cardiac output with exercise i Chest X ray honeycomb pattern ii Open lung biopsy surgery take out the ribs and take a piece of the lung iii Transbronchial biopsy tube goes down to pinch a piece of the iv Bronchoalveolar lavage spray fluid which picks up fibroblasts immuelalveolar cells debris and analyze under a microscope i Smoking cessation avoid environmental exposure ii Anti inflammatory and immunosuppressive agents iii Lung transplant lung i Treatment j Sarcoidosis i Etiology idiopathic ii Systemic disorder affects all tissues 1 Acute common in women 20 30 years old autoimmune that doesn t involve Ab 2 Chronic 30 40 years old iii Trigger causes alveolar macrophage activation iv Pathogenesis 1 Multiple uniform noncaseating epithelioid granulomas spread all over and change function of tissues a Noncasesating solid 2 Multiorgan lymph nodes and lung skin eyes spleen liver kidney bone marrow 3 Fibrotic surrounded by large histiocytes 4 Abnormal T cell function v Clinical manifestations Insidious dyspnea dry cough 1 Malaise fatigue weight loss fever tired weak muscles 2 3 Erythema nodosum macules papules hyperpigmentation subcutaneous nodules subcutaneous nodules red rash 4 Hepatosplenomegaly lymphadenopathy a englarged vi Diagnosis 1 Blood tests a Higher BP from ACE b Leukopenia increased eosinophils anemia c High ESR increased Ca 5 cases high liver enzymes d Anergy decreased sensitivity to specific Ag like Candida and mumps 70 cases false i Anergy w out energy due to leukopenia e High angiotensin converting enzymes active disease 40 80 cases 2 Bronchoalveolar lavage monitor cell content fluid has high lymphocytes and high CD4 CD8 cell ratio 3 Transbronchial lung biopsy noncaseating granulomas definitive diagnosis 4 Chest X ray differentiates stages start with this vii Treatment 1 Corticosteroids and immunosuppressants III Atelectatic Disorders a ARDS secondary to something else i Acute Adult Respiratory Distress Syndrome 1 Associated with other pathophysiological processes 2 125 000 150 000 cases year in U S 30 60 mortality ii Etiology iii Pathogenesis 1 Severe trauma sepsis 40 cases shock 2 Aspiration of gastric acid 30 cases 1 Widespread pulmonary inflammation a Noncardiogenic pulmonary edema leaky pulmonary capillaries b Atelectasia associated with lack of surfactant c Fibrosis inflammatory protein deposition 2 Sever hypoxemia due to intrapulmonary blood shunting a Perfusion of large number of alveoli that are poorly ventilated low ventilation perfusion areas or not ventilated areas of shunt 3 Decrease in lung compliance a Due to surfactant loss inactivation with subsequent increased recoil pressure b Proteinaceous fluid fills alveoli and impairs ventilation 4 Diffuse fluffy alveolar infiltrates iv Clinical manifestations 1 Early a Sudden marked respiratory distress b Mild tachycardia dyspnea low PaO2 c Shallow rapid breathing 2 Late a Tachycardia tachypnea hypotension b Marked restlessness cyanosis c Crackles and ronchi on auscultation use of accessory muscles intercostal and sternal retractions v Diagnosis oxygen levels 1 Hallmark hypoxemia refractory to increased supplemental 2 ABG hypoxia acidosis hypercapnia high carbon dioxide 3 Chest X ray progressive normal to diffuse whiteout 4 Open lung biopsy atelectasis hyaline membranes cell debris interstitial and alveolar edema vi Treatment 1 Treat the sepsis first so the body has time to heal 2 Supportive enhance tissue oxygenation until inflammation resolves Identify and address etiology 3 4 Maintain fluid and electrolyte imbalance fluid administration can produce pulmonary edema 5 Block systemic inflammation 6 Oxygenation IV Neuromuscular Disorders a Poliovirus attacks spinal cord and brainstem motor nerve cells b 8 cases year in U S all related to vaccine administration or unvaccinated immigrants c Clinical manifestations i 95 asymptomatic ii Fever headache sore throat iii Vomiting diarrhea constipation iv Chronic respiratory insufficiency d ALS i Amyotrophic lateral sclerosis ii 2 1 male to female ratio iii Prevalence 5 100 000 iv Degenerative nervous system disease upper and lower motor neurons v Clinical manifestations 1 Progressive muscle weakness and wasting 2 Muscles innervated from spinal and cranial nerves leads to profound respiratory muscle weakness and death e Muscular Dystrophy i Duchenne muscular dystrophy ii Etiology X linked recessive iii Prevalence 1 3500 births iv Clinical manifestations 1 Progressive muscular weakness initially in lower extremities wasting 2 Advanced respiratory muscle involvement in later years 20 30 years results in hypoxia and hypercapnia frequent lung infections f Guillain Barre Syndrome i Acute polyneuritis 1