Condition Lookup

Idiopathic Pulmonary Arterial Hypertension (IPAH)

Category: Pulmonary Vascular Diseases

Sub-category: Pulmonary Hypertension (PH)

Symptoms:
shortness of breath; fatigue; chest pain; dizziness; fainting; swelling in ankles or legs (edema); palpitations; blue discoloration of lips or skin (cyanosis)

Root Cause:
Narrowing and stiffening of pulmonary arteries with no known cause, leading to increased pulmonary vascular resistance and high blood pressure in the lungs.

How it's Diagnosed: videos
Diagnosis is made using echocardiography, right heart catheterization (gold standard), pulmonary function tests, chest X-ray, CT scan, and blood tests to rule out other causes.

Treatment:
Treatment includes medications to lower pulmonary blood pressure, improve exercise capacity, and prevent disease progression; oxygen therapy; and in severe cases, lung transplantation.

Medications:
Medications include phosphodiesterase-5 inhibitors (e.g., sildenafil , tadalafil ), endothelin receptor antagonists (e.g., bosentan , ambrisentan , macitentan ), prostacyclin analogs (e.g., epoprostenol , treprostinil , iloprost ), soluble guanylate cyclase stimulators (e.g., riociguat ), and calcium channel blockers (e.g., nifedipine , diltiazem , only for specific cases).

Prevalence: How common the health condition is within a specific population.
Rare; estimated prevalence is 1-2 cases per million annually, with a higher incidence in women aged 30-50 years.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Female gender, genetic predisposition (BMPR2 gene mutation), and autoimmune diseases.

Prognosis: The expected outcome or course of the condition over time.
Variable; prognosis depends on the severity at diagnosis and response to therapy. Without treatment, survival is 2-3 years, but modern therapies improve long-term outcomes.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Right-sided heart failure, arrhythmias, blood clots, and potential death if untreated.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Specialty: Pulmonology

Category: Pulmonary Vascular Diseases

Sub-category: Pulmonary Hypertension (PH)

Symptoms:
shortness of breath; fatigue; chest pain; dizziness; fainting; swelling in ankles or legs (edema); persistent cough; reduced exercise tolerance

Root Cause:
Chronic obstruction and remodeling of pulmonary arteries due to unresolved blood clots, causing high blood pressure in the lungs.

How it's Diagnosed: videos
Diagnosed using echocardiography, right heart catheterization (gold standard), ventilation-perfusion (V/Q) scan, CT pulmonary angiography, and pulmonary angiography. Blood tests and other imaging may rule out other causes.

Treatment:
Treatment options include pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA), anticoagulation therapy, oxygen therapy, and medications to reduce pulmonary vascular resistance.

Medications:
Medications include anticoagulants (e.g., warfarin , rivaroxaban , apixaban ), soluble guanylate cyclase stimulators (e.g., riociguat ), and prostacyclin analogs or endothelin receptor antagonists in inoperable cases.

Prevalence: How common the health condition is within a specific population.
Rare; affects approximately 3-5% of individuals who have had a pulmonary embolism.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
History of acute pulmonary embolism, inadequate anticoagulation treatment, thrombophilia, splenectomy, or chronic inflammatory diseases.

Prognosis: The expected outcome or course of the condition over time.
With successful pulmonary endarterectomy, prognosis is excellent. In inoperable cases, long-term medical management improves quality of life but prognosis varies.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Right-sided heart failure, arrhythmias, blood clots, and recurrent pulmonary embolism.

Cardiogenic Pulmonary Edema

Specialty: Pulmonology

Category: Pulmonary Vascular Diseases

Sub-category: Pulmonary Edema

Symptoms:
shortness of breath (dyspnea); orthopnea (difficulty breathing while lying flat); paroxysmal nocturnal dyspnea (shortness of breath at night); coughing with frothy pink sputum; rapid breathing (tachypnea); sweating; fatigue and weakness; cyanosis (bluish discoloration of lips or extremities)

Root Cause:
The heart's inability to pump blood effectively leads to increased pressure in the blood vessels of the lungs, causing fluid to leak into the alveoli, resulting in pulmonary edema. This often occurs due to conditions like heart failure, myocardial infarction, or valvular heart disease.

How it's Diagnosed: videos
Physical examination (listening for crackles in the lungs, assessing for signs of heart failure). Chest X-ray (shows signs of pulmonary edema). Echocardiogram (to assess heart function). BNP (B-type natriuretic peptide) test (elevated in heart failure). Arterial blood gas (ABG) test (to assess oxygen levels). Pulmonary artery catheterization (in severe cases).

Treatment:
Oxygen therapy to improve oxygen levels in the blood. Diuretics (e.g., furosemide) to reduce fluid overload. Inotropes (e.g., dobutamine) to improve heart function. Vasodilators (e.g., nitroglycerin) to reduce preload and afterload on the heart. Mechanical ventilation or non-invasive positive pressure ventilation (if severe). Treatment of underlying cause (e.g., coronary artery bypass, valve repair, or treatment for heart failure).

Medications:
Diuretics (e.g., furosemide ) to reduce fluid retention and pulmonary edema. Inotropes (e.g., dobutamine ) to improve heart function in cases of heart failure. Vasodilators (e.g., nitroglycerin ) to relieve heart stress by dilating blood vessels. ACE inhibitors or ARBs to manage underlying hypertension or heart failure.

Prevalence: How common the health condition is within a specific population.
Cardiogenic pulmonary edema is common in individuals with chronic heart failure, acute myocardial infarction, or valvular diseases. It is responsible for a significant portion of hospital admissions for acute heart failure.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
History of heart disease (e.g., coronary artery disease, heart failure). Hypertension. Valvular heart disease (e.g., aortic or mitral valve stenosis or regurgitation). Arrhythmias (e.g., atrial fibrillation). Myocardial infarction (heart attack). Obesity. Diabetes. Smoking.

Prognosis: The expected outcome or course of the condition over time.
The prognosis depends on the severity of the heart failure, the underlying cause, and the timeliness of treatment. With appropriate treatment, many individuals can recover, but some may experience chronic heart failure or recurring episodes of pulmonary edema.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Respiratory failure. Cardiogenic shock. Renal failure due to poor perfusion. Arrhythmias. Recurrent episodes of pulmonary edema. Long-term heart failure.

Non-Cardiogenic Pulmonary Edema

Specialty: Pulmonology

Category: Pulmonary Vascular Diseases

Sub-category: Pulmonary Edema

Symptoms:
shortness of breath (dyspnea); tachypnea (rapid breathing); hypoxia (low oxygen levels); cyanosis (bluish lips and extremities); crackles heard on lung auscultation; coughing, often without sputum; fatigue and weakness

Root Cause:
Non-cardiogenic pulmonary edema occurs due to damage to the capillary endothelium, causing increased permeability of the pulmonary capillaries. This allows fluid to leak into the alveoli without heart failure involvement. Common causes include acute respiratory distress syndrome (ARDS), inhalation injuries, infections, or trauma.

How it's Diagnosed: videos
Chest X-ray (often showing diffuse bilateral infiltrates). Arterial blood gas (ABG) to assess oxygenation. Echocardiogram (to rule out cardiac causes). Pulmonary function tests (to assess lung injury). Clinical history (trauma, infection, or other systemic causes)

Treatment:
Oxygen therapy to support breathing and oxygenation. Mechanical ventilation or non-invasive positive pressure ventilation. Treating the underlying cause (e.g., antibiotics for infections, corticosteroids for inflammation). Diuretics (if fluid overload is present). Supportive care (nutritional support, fluid management).

Medications:
Corticosteroids (e.g., methylprednisolone ) may be used to reduce lung inflammation, particularly in ARDS. Antibiotics (e.g., ceftriaxone ) for infections like pneumonia that contribute to non-cardiogenic pulmonary edema. Diuretics (e.g., furosemide ) if fluid overload is present, although not typically used in non-cardiogenic pulmonary edema unless fluid retention is significant.

Prevalence: How common the health condition is within a specific population.
Non-cardiogenic pulmonary edema is less common than cardiogenic pulmonary edema but is associated with conditions like ARDS and severe trauma, which can occur in various clinical settings.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Acute respiratory distress syndrome (ARDS). Trauma (e.g., head injury, chest trauma, burns). Inhalation of toxic substances (e.g., smoke, chemicals). Severe infections (e.g., pneumonia, sepsis). Acute pancreatitis. Near-drowning. Aspiration of gastric contents.

Prognosis: The expected outcome or course of the condition over time.
Prognosis is often dependent on the underlying cause. In cases related to ARDS or trauma, outcomes can vary greatly, with some individuals recovering with minimal long-term effects, while others may experience persistent respiratory issues.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Respiratory failure. Long-term lung damage or fibrosis. Persistent hypoxia. Organ failure due to severe systemic inflammation or infection. Chronic respiratory issues (e.g., pulmonary fibrosis).

Granulomatosis with Polyangiitis (GPA, formerly Wegener’s)

Specialty: Pulmonology

Category: Pulmonary Vascular Diseases

Sub-category: Vasculitis with Pulmonary Involvement

Symptoms:
nasal congestion; sinus pain or bleeding; cough (sometimes with blood); shortness of breath; fatigue; weight loss; joint pain

Root Cause:
GPA is an autoimmune disease where the immune system attacks blood vessels, leading to inflammation and damage to various organs, particularly the lungs, kidneys, and upper respiratory tract.

How it's Diagnosed: videos
Blood tests (e.g., ANCA – antineutrophil cytoplasmic antibodies). Imaging (chest X-ray, CT scans of the chest). Biopsy of affected tissue (usually lung or kidney).

Treatment:
Immunosuppressive therapy to control inflammation (such as cyclophosphamide, rituximab). Glucocorticoids (steroids). Plasmapheresis for severe cases (removal of circulating antibodies).

Medications:
Cyclophosphamide (chemotherapy agent, immunosuppressant). Rituximab (monoclonal antibody, immunosuppressant). Prednisone (corticosteroid, anti-inflammatory). Methotrexate (immunosuppressant, chemotherapy agent). Trimethoprim-sulfamethoxazole (antibiotic for prevention of infections).

Prevalence: How common the health condition is within a specific population.
Rare, with an estimated incidence of 3 to 12 cases per million people per year.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Genetic predisposition, environmental factors (such as exposure to certain chemicals or infections), and smoking.

Prognosis: The expected outcome or course of the condition over time.
With early diagnosis and appropriate treatment, many patients achieve remission. However, it can be life-threatening without treatment and relapses are common.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Kidney failure. Lung damage. .Respiratory failure. Infections due to immunosuppressive therapy. Heart complications (in rare cases).

Churg-Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis)

Specialty: Pulmonology

Category: Pulmonary Vascular Diseases

Sub-category: Vasculitis with Pulmonary Involvement

Symptoms:
asthma or worsening asthma symptoms; eosinophilia (elevated eosinophil count in the blood); sinusitis; fatigue; weight loss; muscle and joint pain; skin rashes or lesions

Root Cause:
An autoimmune vasculitis that causes inflammation of blood vessels, particularly affecting the lungs and the peripheral nerves. It’s often associated with severe asthma and eosinophilia.

How it's Diagnosed: videos
Blood tests (eosinophil count, ANCA). Chest X-ray or CT scan. Biopsy (skin or lung).

Treatment:
Immunosuppressive medications (such as cyclophosphamide or rituximab). Corticosteroids (prednisone) to control inflammation. Bronchodilators for asthma management.

Medications:
Cyclophosphamide (chemotherapy agent, immunosuppressant). Rituximab (monoclonal antibody, immunosuppressant). Prednisone (corticosteroid, anti-inflammatory). Methotrexate (immunosuppressant). Inhalers (bronchodilators for asthma management).

Prevalence: How common the health condition is within a specific population.
Rare, with an estimated incidence of 1 to 3 cases per million people per year.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Asthma, atopic conditions (e.g., hay fever), and genetic factors.

Prognosis: The expected outcome or course of the condition over time.
With appropriate treatment, many patients experience remission. However, the condition can be life-threatening without prompt intervention.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Heart damage (especially in severe cases). Kidney damage. Respiratory failure due to lung involvement. Nerve damage or neuropathy.