Sarcoidosis Flashcards
What is sarcoidosis?
Systemic granulomatous disease of unknown origin that is highly associated with lung involvement but can affect all organs.
What is the typical age of onset for sarcoidosis?
Before 50 years old, with peak incidence in 20-40 years old.
Which racial and ethnic groups are most affected by sarcoidosis?
All racial and ethnic groups, but most prevalent in individuals with north-european ancestry.
Is sarcoidosis more common in males or females?
More common in females than males.
How does socioeconomic status relate to sarcoidosis severity?
Lower socioeconomic status is associated with more severe sarcoidosis.
What is the familial tendency of sarcoidosis?
Clusters in families, more likely to occur in individuals with family members who have sarcoidosis.
What triggers the immune response in sarcoidosis?
An unknown antigen presented on antigen-presenting cells (APC).
Which cells are primarily involved in the immune response of sarcoidosis?
T-helper cells and cytokines such as macrophage-derived TNF-alpha, IL-12, IL-18.
What forms as a result of the inflammatory response in sarcoidosis?
Sarcoid epithelioid granuloma.
What role do T17 helper cells play in sarcoidosis?
They produce IL-17, which inhibits T-regulatory (Treg) cells.
What is a Treg cell?
Immunosuppressive cell that prevents autoimmunity by maintaining self-tolerance.
Which organs are most commonly affected by sarcoidosis?
Lungs, eyes, and skin.
What are the pulmonary manifestations of sarcoidosis?
Mediastinal and symmetrical hilar lymphadenopathy, dry cough, dyspnea, nonspecific chest pain.
What is parenchymal involvement in sarcoidosis characterized by?
Nodules, fibrosis, and traction bronchiectasis, with crackles on auscultation.
What symptoms may indicate airway involvement in sarcoidosis?
Asthma-like symptoms.
What are potential cardiac manifestations of sarcoidosis?
Sudden death, arrhythmias, cardiomyopathy.
What neurological manifestations can occur with sarcoidosis?
Seizures, cranial nerve palsies, aseptic meningitis.
What are the ophthalmic manifestations associated with sarcoidosis?
Uveitis, vision loss, dry eyes.
What bone-related symptoms may occur in sarcoidosis?
Arthralgia causing difficulty walking, mostly affecting lower limb joints and fingers.
What dermatological manifestation is commonly seen in sarcoidosis?
Erythema nodosum.
What renal issue can arise from sarcoidosis?
Kidney stones due to hypercalcaemia.
What is Lofgren’s syndrome?
Acute form of sarcoidosis presenting with classic triad of erythema nodosum, bi-hilar lymphadenopathy, and polyarthralgia with fever.
How long does Lofgren’s syndrome typically resolve?
Within 6-24 months.
What is the classification of pulmonary sarcoidosis based on chest x-ray and HRCT?
Pulmonary sarcoidosis is classified into stages 0 to IV based on chest x-ray and HRCT findings.
What are the findings in Stage 0 of pulmonary sarcoidosis?
No abnormal findings to suggest pulmonary sarcoidosis.
What are the findings in Stage I of pulmonary sarcoidosis?
Hilar and mediastinal lymphadenopathy with clear lungs.
What are the findings in Stage II of pulmonary sarcoidosis?
- Hilar and mediastinal lymphadenopathy 2. Reticular opacities/shadowing due to parenchymal infiltration.
What are the findings in Stage III of pulmonary sarcoidosis?
Lung nodules due to granulomas.
What are the findings in Stage IV of pulmonary sarcoidosis?
Pulmonary fibrosis with bilateral reticular opacities, shaggy/unclear heart borders, lung volume loss, and honeycombing.
What is the significance of reticular opacities in Stage IV?
Bilateral reticular opacities are most prominent around lung bases.
What is observed in Stage IV regarding lung nodules?
Normal-sized nodes with lung fibrosis.
What investigations are done for sarcoidosis?
FBC: Check for anaemia or lymphopenia
U&E: Check renal function
LFTS
Serum angiotensin converting enzyme (SACE): Elevated levels in granulomatous-inflammatory conditions such as sarcoidosis
Histology: Non-caseating granulomas
What is the management approach for sarcoidosis?
Most patients don’t need treatment but it’s considered in patients who meet the requirements of Well’s law.
What are the two main principles of Well’s law of sarcoidosis treatment?
- Prevent organ damage or dangerous disease
- To improve quality of life
What is the first-line treatment for sarcoidosis?
Prednisolone
What are the second-line treatments for sarcoidosis?
MTX, azathioprine
Azathioprine is indicated for neurosarcoidosis and has a higher infection rate than MTX.
What are the side effects of second-line treatments for sarcoidosis?
Cytopenias, gastrointestinal effects, LFT derangement
What are the third-line treatments for sarcoidosis?
Mycophenolate Mofetil, hydroxychloroquine, anti-TNF biologics
MMF is indicated for neurosarcoidosis but is better tolerated than azathioprine.
What is the indication for hydroxychloroquine in sarcoidosis?
Indicated for musculoskeletal manifestations and hypercalcemia
Side effects include optic neuritis.
What is the indication for anti-TNF biologics in sarcoidosis?
Indicated for ophthalmic manifestations and neurosarcoidosis
Side effects include paradoxical granulomatous disease and sepsis.
