Condition Lookup

Idiopathic Pulmonary Fibrosis (IPF)

Sub-category: Interstitial Lung Diseases (ILD)

Symptoms:
shortness of breath (dyspnea); persistent dry cough; fatigue; unexplained weight loss; clubbing of fingers; chest discomfort

Root Cause:
Chronic scarring (fibrosis) of lung tissue with no known cause, leading to stiff and thickened lungs that impair oxygen exchange.

How it's Diagnosed: videos
High-resolution computed tomography (HRCT), pulmonary function tests, lung biopsy (if necessary), arterial blood gas analysis, and exclusion of other ILD causes.

Treatment:
Antifibrotic therapy, pulmonary rehabilitation, oxygen therapy, lung transplantation in advanced cases, and supportive care.

Medications:
Pirfenidone (an antifibrotic agent) and nintedanib (a tyrosine kinase inhibitor) are prescribed to slow disease progression. Both are classified as antifibrotic medications.

Prevalence: How common the health condition is within a specific population.
Approximately 13-20 per 100,000 people globally, with higher prevalence in individuals over 60 years old.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Older age, male gender, smoking history, family history of IPF, and exposure to environmental/occupational pollutants.

Prognosis: The expected outcome or course of the condition over time.
Progressive and incurable; median survival is 3-5 years after diagnosis. Early intervention with antifibrotics and lung transplant can improve outcomes.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Respiratory failure, pulmonary hypertension, increased susceptibility to lung infections, and increased risk of lung cancer.

Sarcoidosis

Specialty: Pulmonology

Category: Restrictive Lung Diseases

Sub-category: Interstitial Lung Diseases (ILD)

Symptoms:
shortness of breath; dry cough; fatigue; fever; chest pain; red or swollen patches on the skin; enlarged lymph nodes; eye irritation or vision problems

Root Cause:
Formation of granulomas (clusters of inflammatory cells) in multiple organs, particularly the lungs and lymph nodes, leading to inflammation and tissue damage.

How it's Diagnosed: videos
Chest X-ray, HRCT, pulmonary function tests, biopsy showing non-caseating granulomas, blood tests (ACE levels, calcium), and exclusion of other granulomatous diseases.

Treatment:
Corticosteroids (mainstay of treatment), immunosuppressive agents (e.g., methotrexate), and symptom management.

Medications:
Prednisone (a corticosteroid) is commonly prescribed. Immunosuppressants like methotrexate , azathioprine , or mycophenolate mofetil may be used for steroid-sparing effects.

Prevalence: How common the health condition is within a specific population.
Affects approximately 10-40 per 100,000 people, more common in African Americans and women.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Genetic predisposition, environmental exposures, and immune system dysregulation.

Prognosis: The expected outcome or course of the condition over time.
Many cases resolve spontaneously; some develop chronic disease, with about 10-15% of cases leading to permanent lung damage.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Pulmonary fibrosis, pulmonary hypertension, heart involvement (sarcoid cardiomyopathy), and organ failure.

Cryptogenic Organizing Pneumonia (COP)

Specialty: Pulmonology

Category: Restrictive Lung Diseases

Sub-category: Interstitial Lung Diseases (ILD)

Symptoms:
cough; fever; fatigue; shortness of breath; weight loss; chills; muscle aches

Root Cause:
Inflammation and fibrosis of the small airways and alveoli (air sacs) in the lungs, leading to the formation of fibrous tissue plugs in the bronchioles and alveolar ducts. The cause is unknown, but it may follow a respiratory infection or be associated with autoimmune diseases.

How it's Diagnosed: videos
HRCT showing characteristic pattern of consolidation or ground-glass opacities, lung biopsy showing organizing pneumonia with fibrous tissue, and exclusion of other causes.

Treatment:
Corticosteroids (prednisone) are the first-line treatment, with longer durations in cases of relapse.

Medications:
Prednisone (a corticosteroid) is prescribed to reduce inflammation. Immunosuppressants like azathioprine may be used in cases of steroid resistance or relapse.

Prevalence: How common the health condition is within a specific population.
Rare, estimated incidence is about 1 per 100,000 people per year.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Previous respiratory infections, exposure to certain drugs, autoimmune conditions, or environmental factors like smoking.

Prognosis: The expected outcome or course of the condition over time.
Generally favorable with treatment; many patients recover with steroid therapy, but relapse can occur in some cases.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Relapse of disease, development of pulmonary fibrosis in severe cases, and chronic restrictive lung disease if untreated.

Nonspecific Interstitial Pneumonia (NSIP)

Specialty: Pulmonology

Category: Restrictive Lung Diseases

Sub-category: Interstitial Lung Diseases (ILD)

Symptoms:
shortness of breath; dry cough; fatigue; fever; unexplained weight loss; muscle aches

Root Cause:
Inflammation and fibrosis of the lung interstitial tissue without a clear cause, often associated with autoimmune diseases such as rheumatoid arthritis or scleroderma.

How it's Diagnosed: videos
HRCT showing ground-glass opacities, pulmonary function tests showing restrictive pattern, and lung biopsy showing a homogeneous pattern of inflammation and fibrosis.

Treatment:
Corticosteroids (prednisone) are commonly used to reduce inflammation, with immunosuppressants like methotrexate or cyclophosphamide used for autoimmune-related cases.

Medications:
Prednisone (a corticosteroid) is used to control inflammation. Methotrexate , azathioprine , or mycophenolate mofetil may be used for autoimmune-related cases.

Prevalence: How common the health condition is within a specific population.
Relatively rare; no exact global prevalence but accounts for about 10-20% of idiopathic interstitial pneumonias.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Autoimmune diseases, environmental exposures, and genetic predispositions.

Prognosis: The expected outcome or course of the condition over time.
Generally better than IPF but still associated with variable progression. Patients may respond well to corticosteroids, but some may develop persistent or progressive fibrosis.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Pulmonary fibrosis, respiratory failure, and associated autoimmune disease complications.

Acute Interstitial Pneumonia (AIP)

Specialty: Pulmonology

Category: Restrictive Lung Diseases

Sub-category: Interstitial Lung Diseases (ILD)

Symptoms:
acute shortness of breath; cough; fever; rapid breathing; hypoxia; general malaise

Root Cause:
Rapid, severe inflammation and fibrosis of the lung interstitial tissue, often progressing to acute respiratory failure. The cause is often unknown, but it may follow viral infections or be associated with autoimmune diseases.

How it's Diagnosed: videos
HRCT showing bilateral ground-glass opacities and consolidations, biopsy revealing diffuse alveolar damage, and exclusion of other causes of acute lung injury.

Treatment:
Acute cases are treated with high-dose corticosteroids and sometimes immunosuppressants. Supportive care, including mechanical ventilation, may be required in severe cases.

Medications:
Prednisone (a corticosteroid) for inflammation; other immunosuppressive agents may be considered depending on the cause or severity.

Prevalence: How common the health condition is within a specific population.
Extremely rare; fewer than 1 per 100,000 people are affected.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Recent viral infections, underlying autoimmune diseases, and smoking.

Prognosis: The expected outcome or course of the condition over time.
Poor in acute cases; up to 50% of patients may require lung transplantation. Mortality is high due to respiratory failure.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Respiratory failure, pulmonary fibrosis, secondary infections, and multi-organ failure.

Lymphangioleiomyomatosis (LAM)

Specialty: Pulmonology

Category: Restrictive Lung Diseases

Sub-category: Interstitial Lung Diseases (ILD)

Symptoms:
shortness of breath; chest pain; cough; fatigue; pneumothorax; hemoptysis; abdominal pain

Root Cause:
Abnormal growth of smooth muscle cells in the lungs and lymphatic system, leading to cystic destruction of the lung tissue and airway obstruction. Most common in women of reproductive age and often associated with tuberous sclerosis complex.

How it's Diagnosed: videos
HRCT showing characteristic cystic lesions, pulmonary function tests showing restrictive lung pattern, and genetic testing for tuberous sclerosis (if suspected).

Treatment:
No definitive cure; treatment focuses on symptom management, including oxygen therapy and management of pneumothorax. In some cases, lung transplantation may be considered.

Medications:
Rapamycin (sirolimus ), an immunosuppressant, may be used to slow disease progression, particularly in tuberous sclerosis-associated LAM.

Prevalence: How common the health condition is within a specific population.
Rare; affects about 1-2 women per 1,000,000 annually.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Genetic predisposition, specifically in individuals with tuberous sclerosis complex.

Prognosis: The expected outcome or course of the condition over time.
Progressive with variable severity; lung transplantation may be necessary in advanced cases.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Pneumothorax, pulmonary hypertension, respiratory failure, and renal tumors associated with tuberous sclerosis.

Pulmonary Langerhans Cell Histiocytosis (PLCH)

Specialty: Pulmonology

Category: Restrictive Lung Diseases

Sub-category: Interstitial Lung Diseases (ILD)

Symptoms:
cough; shortness of breath; chest pain; fatigue; fever; unexplained weight loss

Root Cause:
Abnormal proliferation of Langerhans cells (a type of immune cell) in the lungs, causing granulomatous inflammation and tissue damage, most often in heavy smokers.

How it's Diagnosed: videos
HRCT showing cystic and nodular lesions, biopsy revealing Langerhans cells and characteristic granulomas.

Treatment:
Smoking cessation, corticosteroids for inflammation, and chemotherapy or immunotherapy in severe cases.

Medications:
Prednisone (corticosteroid) for inflammation; other immunosuppressants like methotrexate may be used for refractory disease.

Prevalence: How common the health condition is within a specific population.
Rare; primarily affects young adults, with a higher prevalence in smokers.

Risk Factors: Factors or behaviors that increase the likelihood of developing the condition.
Smoking, possibly occupational exposures to certain chemicals or dust.

Prognosis: The expected outcome or course of the condition over time.
Often self-limiting in mild cases, but severe disease can progress to respiratory failure and fibrosis.

Complications: Additional problems or conditions that may arise as a result of the original condition.
Pulmonary fibrosis, pneumothorax, and secondary infections.