Sarcoidosis Flashcards
What is sarcoidosis?
Sarcoidosis is a multisystem chronic inflammatory condition characterised by the formation of non-caseating epithelioid granulomatous lumps at various sites in the body.
It has a predilection for the lungs and thoracic cavity; however, there are many different manifestations that may cause a great deal of diagnostic difficulty.
What are the most commonly affected areas by sarcoidosis?
After the thorax, the skin and eyes are most commonly affected, followed by the liver (not usually clinically relevant), heart and nervous system.
At what ages does sarcoidosis mostly commonly appear?
mid-20s to mid-40s
How do people with sarcoidosis usually present?
Common symptoms, which tend to be vague, include fatigue, lack of energy, weight loss, joint aches and pains, arthritis, dry eyes, shortness of breath, blurry vision, cough, skin lesions
What are the lung symptoms associated with sarcoidosis?
Patients may present with dry cough, fever and dyspnoea accompanied by chest discomfort.
The lungs are affected in more than 90% of people with sarcoidosis.
Pulmonary function studies are abnormal in many patients with sarcoidosis but there is no diagnostic pattern.
What are the skin symptoms associated with sarcoidosis?
Papules may be seen on the face and resembling rosacea or maculopapular rashes on the body or extremities. Brownish-red infiltrative plaques on the extremities and trunk may be present (very similar in appearance to plaque psoriasis).
Erythema nodosum on the legs is a relatively common feature
What are the eye symptoms associated with sarcoidosis?
This is affected in >20% of cases, most frequently as a granulomatous uveitis.
What are the neuro symptoms associated with sarcoidosis?
Bell’s palsy and lymphocytic meningitis are common manifestations of neurological involvement but diabetes insipidus is also seen.
Facial numbness, dysphagia, hoarseness, headache, visual field defects, polydipsia, hearing impairment, lesions of cranial nerves VII, VIII, IX and X
What signs are associated with sarcoidosis?
- Lupus pernio
- Uveitis, dry eyes
- Painful joints
- Hepatomegaly and splenomegaly
- Crackles, but only in advanced interstitial lung disease
What investigations would you do if you suspected sarcoidosis?
Histology is key
No diagnostic test, diagnosis of exclusion
FBC may show raised white count/eosinophilia or lymphopenia. Anaemia may be seen.
ESR is often raised
Plain CXR may show bilateral hilar or paratracheal lymphadenopathy. High-resolution CT scanning is often used to detect interstitial lung disease.
What histological finding would support a diagnosis of sarcoidosis?
Granulomatous inflammation, systemic
How would you stage pulmonary involvement in sarcoidosis?
CXR findings
Stage 0 - normal findings on chest radiograph.
Stage I - bilateral hilar lymphadenopathy
Stage II - bilateral hilar adenopathy with pulmonary infiltrates
Stage III - parenchymal infiltrates without hilar adenopathy.
Stage IV - parenchymal involvement turns into volume loss (pulmonary fibrosis) and there may be other features (cavitations, calcifications, hilar retraction, bullae, cysts and emphysema).
How would you manage sarcoidosis?
About half of patients with sarcoidosis will need systemic therapy for their disease.
Oral glucocorticoids are the standard first-line treatment for sarcoidosis (be careful, monitor!)
Antimetabolites are used as alternatives to steroids
Consideration of prophylaxis against osteoporosis with bisphosphonate drugs is recommended but calcium and vitamin D are usually avoided due to the risk of hypercalcaemia in sarcoidosis.
What is the prognosis for sarcoidosis?
Although many patients have sarcoidosis which resolves spontaneously, a significant proportion of patients have chronic or progressive disease with resultant morbidity. About 20% of patients have permanent clinical symptoms because of irreversible fibrosis, mainly pulmonary fibrosis.
What causes sarcoidosis?
The exact cause of sarcoidosis is not known.
The current working hypothesis is, in genetically susceptible individuals, sarcoidosis is caused through alteration to the immune response after exposure to an environmental, occupational, or infectious agent
