Sarcoidosis Flashcards
Characteristics of sarcoidosis
- Sarcoidosis is a multisystem disorder characterized by NONCASEATING GRANULOMATOUS inflammation.
- It is classified as either acute or chronic; chronic sarcoidosis is not necessarily preceded by acute sarcoidosis.
- Acute sarcoidosis has an abrupt onset with constitutional symptoms (e.g., fever, malaise) as well as cough, dyspnea, anterior uveitis, erythema nodosum, and arthralgia, and it is self-limiting after a few years.
- Chronic sarcoidosis has an insidious onset and is often asymptomatic in its early stages. It primarily affects the lungs, although other systemic manifestations are also possible. The first symptoms of chronic sarcoidosis usually include exertional dyspnea and a dry cough with mild rales on pulmonary examination.
- A chest x-ray may show parenchymal disease with bilateral hilar lymphadenopathy, but these features are not always evident.
- Biopsy is the gold standard for diagnosis.
- The most common histopathological finding is noncaseating granulomas
with giant cells. - Glucocorticoid therapy is indicated with disease progression or if certain organs, such as the eyes or heart, are affected. While spontaneous remission rates are high during the early stages of sarcoidosis, irreversible lung fibrosis may develop as the disease recurs or progresses.
Clinical features of acute sarcoidosis
- Sudden onset and remits spontaneously within 2 years
- Progression to chronic is rare
- General: fever, malaise, lack of appetite, weight loss
- Pulmonary: dyspnoea, cough, chest pain
- Extrapulmonary: arthritis, anterior uveitis, erythema nodosum
Clinical features of chronic sarcoidosis
Pulmonary (most common)
- Often asymptomatic in early stages
- Interstitial fibrosis: cough, exertional dyspnoea, mild rales
Extrapulmonary
- Peripheral LN involvement
- Ocular: granulomatous uveitis, blurred vision
- Skin:
lupus pernio - pathognomonic extensive purple skin lesions on the nose/cheeks chin
Scar sarcoidosis
- Heart: restrictive cardiomyopathy
- Symmetric arthritis, hepatosplenomegaly, nephrolithiasis due to hypercalcaemia
Features of sarcoidosis summarised!
Features of sarcoidosis are GRUELING
- Granulomatous
- Arthritis - acute and chronic arthritis particularly involves the ankles and knees
- Uveitis
- Erythema nodosum
- Lymphadenopathy (bilateral hilar lymphadenopathy)
- Interstitial fibrosis
- Negative TB
- Gammaglobulinaemia
What is lofgren syndrome?
Acute presentation of fever and the following triad of symptoms
- Migratory polyarthritis: symmetrical arthritis that primarily affects the ankles
- Erythema nodosum: affects the extensor surface of the lower legs
- Bilateral hilar lymphadenopathy
Tx: usually self-limited, NSIADs, colchicine or low dose pred
Methotrexate, high dose pred or TNFa inhibitor like adalimumab are reserved for patients with chronic, organ damaging forms of sarcoidosis.
What is Heerfordt syndrome?
Chronic clinical presentation with fever and triad of symptoms
- Parotitis
- Uveitis
- Facial palsy
Investigations for sarcoidosis
- CXR: hilar lymphadeopathy
- HRCT
LAB
Acute sarcoidosis:
- High CRP
Chronic Sarcoidosis
- Elevated calcium due to elevated levels of 1,25 vitamin D3
- Elevated ACE: used to monitor disease activity and therapy
- Elevated interleukin 2 receptor: correlate with disease activity
- Elevated alkaline phosphatase
- Reduced CD4 T cells: T helper cells are consumed during granuloma formatoin –> low CD4 levels in SERUM but HIGH in bronchoalveolar lavage
- Elevated IgG
- Hypercalciuria
Bronchosopy + biopsy = gold standard from lung tissue or LN
- Non caseating granulomas with giant cells
Bronchoalveolar lavage - elevated CD4/CD8 ratio
Treatment for sarcoidosis
Isolated Pulmonary Sarcoidosis
- No treatment
- Often asymptomatic, non progressive and high rate of spontaneous remission
Symptomatic or Extrapulmonary Sarcoidosis
- 1st Line: steroids
- 2nd line: alternative immunosuppressive therapy (eg; methotrexate or azathioprine)
- Antimalarial drugs - chloroquine, hydroxychloroquine
- Last resort: lung transplant
- NSIAD always indicated for symptom relief
Complications of sarcoidosis
- Increased risk of malignancy (lung and malignant lymphoma)
- Bronchiectasis
- Lung fibrosis
- Chronic renal failure
