Respiratory Diseases

▪. Pulmonary arteriovenous fistulas have congenital and hereditary etiology, and patients are at risk for life-threatening rupture requiring surgery. ▪. Wegener's granulomatosis can affect any organ system, although renal and pulmonary involvement is most common; men ages 40 to 50 are at increased risk. ▪. Lymphomatoid granulomatosis affects cardiopulmonary, neurologic, and myeloproliferative systems; may result from opportunistic infection, and frequently progresses to lymphoma; men age 50 to 60 are at increased risk. Spontaneous remission occurs in some cases; mortality is 60% to 90% at 5 years. ▪. Churg-Strauss syndrome is usually associated with long-standing asthma, with men and women affected equally, and can affect any organ system; major cause of death is cardiac related. ▪. Primary pulmonary hypertension is a diagnosis of exclusion; women are affected twice as likely as men; right-to-left shunt may occur in 30%, secondary to patent foramen ovale; hypoxia with resultant heart failure is typical cause of death. ▪. Cystic fibrosis is an autosomal recessive disease, eventually fatal, with increased risk for airway obstruction, fluctuating pulmonary function, and chronic hypoxia; risk for spontaneous pneumothorax is 20%. ▪. Bronchiolitis obliterans organizing pneumonia is a pulmonary obstructive disease that may be reversible and usually resolves spontaneously. ▪. Idiopathic pulmonary hemosiderosis is associated with autoimmune disorders; patients have recurrent hemorrhage, pulmonary fibrosis, restrictive lung disease, and pulmonary hypertension, with some cases of spontaneous remission. ▪. Chronic eosinophilic pneumonia may be preceded by adult-onset asthma; women are at increased risk; prognosis is good. ▪. Goodpasture's syndrome is a genetic autoimmune disorder involving the pulmonary and renal systems. ▪. Pulmonary alveolar proteinosis, a lipoprotein-rich accumulation in alveoli, has three forms: congenital, decreased alveolar macrophage activity, and idiopathic; some cases of spontaneous remission occur. ▪. Sarcoidosis may affect any organ system; African American, northern European, and females are at greater risk; many patients are asymptomatic. ▪. Systemic lupus erythematosus may affect any organ system; women of childbearing age are at increased risk. ▪. Idiopathic pulmonary fibrosis is a rare interstitial lung disease, with smokers at increased risk for pulmonary malignancy; survival is usually 2 to 3 years from diagnosis; no effective treatment exists, with lung transplant the only therapeutic option. ▪. Acute respiratory distress syndrome (ARDS) is associated with underlying critical illness or injury, developing acutely in 1 to 2 days; mortality is 25% to 35%. ▪. Pulmonary histiocytosis X is an interstitial lung disease associated with cigarette smoking and an unpredictable course; some spontaneous remission occurs. ▪. Lymphangioleiomyomatosis involves progressive deterioration of lung function, associated with tuberous sclerosis and exacerbated by pregnancy, with women at increased risk; possible spontaneous pneumothorax and chylothorax; death usually results from respiratory failure. ▪. Ankylosing spondylitis is a genetic inflammatory process resulting in fusion of axial skeleton and spinal deformities, with men at increased risk; radiologic bamboo spine, sacral to cervical progression, and restrictive lung disease with high reliance on diaphragm; extraskeletal manifestations may occur. ▪. Kyphosis (exaggerated anterior flexion) and scoliosis (lateral rotational deformity) are spinal/rib cage deformities with idiopathic, congenital, or neuromuscular etiology; corrective surgery done if Cobb thoracic angle >50% lumbar angle >40%. ▪. Bleomycin is an antineoplastic antibiotic used in combination chemotherapy, with no myelosuppressive effect; toxicity can cause life-threatening pulmonary fibrosis. ▪. Influenza A is highly infectious, presenting with flulike symptoms and possible progression to ARDS; human-to-human exposure is through droplets or contaminated surfaces, with high risk for infants, children, pregnancy, chronically ill, or renal replacement therapy patients. No prophylactic treatment exists; treat patients with high index of suspicion without definitive testing; rRT-PCR and viral cultures are sensitive for pandemic H1N1 strain. ▪. Severe acute respiratory syndrome (SARS) is highly infectious, transmitted by coronavirus with human-to-human exposure via droplets or surfaces, and may progress to ARDS. ▪. Echinococcal disease of lung is from canine tapeworm, transmitted by eggs from feces; rupture of cyst may result in anaphylactic reaction or spread of disease to other organs; children are at increased risk. No transthoracic needle aspiration is done; surgery is only option.