Usually no treatment is necessary. Spontaneous remission occurs in
- 90% of patients with only bilateral hilar lymphadenopathy (ie stage 1 pulmonary disease)
- 50% of patients with bilateral hilar lymphadenopathy and pulmonary shadowing (stage 2).
In general, decision to initiate therapy or not can be based on two principles.
- the first one is when the organ functions are affected which may result in severe and/or irreversible organ dysfunction or become life threatening.
- the second principle is when patients suffer from serious and unacceptable complains which reduces quality of life (but not dangerous to the patient) (2).
The US guidelines and BTS guidelines state that treatment decision should reflect a balance between the risks of using corticosteroids (most common therapy) and the potential benefits (1).
- short term use of regular paracetamol and NSAIDs may be helpful in painful erythema nodosum (1)
- treatment is not indicated for patients with
- asymptomatic stage I disease
- asymptomatic stable stage II or III disease who have only mildly abnormal lung function
- oral steroids may be useful in patients with stage 2 or 3 disease who have moderate to severe or progressive symptoms or changes on chest radiography
Oral corticosteroids remain the principle treatment (1). Long term high dose corticosteroid use and a burst-and-taper approach (used for asthma) should be avoided (3)
- UK guidelines recommend an initial treatment of prednisolone, 0.5 mg/kg/day for four weeks which is decreased gradually over the next six months ideally to a maintenance dose of around 10mg or less
- Treatment duration may vary but usually used for a period of 6 -24 months
- an oral bisphosphonate (empirically) is used to minimize corticosteroid induced bone loss (1)
- inhaled corticosteroids are not useful as an initial treatment method or for maintenance therapy. They may be considered in patients with intractable cough to control the symptom (1).
Patients who are unresponsive to steroids or if steroids cannot be reduced or stopped (patients who need > 10mg/day to control the disease), a steroid sparing agent can be added with the aim of subsequently reducing the dosage to 10mg/day or less
- commonly used agents include (although there are no high quality evidence which supports the use of these drugs) - hydroxychloroquine, methotrexate, azathioprine, cyclophosphamide.
- due to the high toxicity these drugs should be prescribed under expert supervison (1)
A tumor necrosis factor inhibitor can be used in patients who have failed a trial of the above agents. e.g. – infliximab (commonly used), etanercept, adalimumab (1).
In patients with disfiguring skin lesions then chloroquine may be used as a steroid sparing agent.
Severe arthralgia or erythema nodosum may respond to aspirin or possibly prednisolone in a modest dosage for two to three weeks.
Progression of the disease should be monitored by serial lung function tests, chest radiology and ACE levels.
Note:
- clinicians should keep in mind that although corticosteroids and other immunosuppressive or immunomodulatory drugs are effective in treatment of the disease, they are not curative and relapses are seen frequently once the dosage is decreased (3)
- corticosteroids in sarcoidosis
- although clear evidence is lacking on the effective dose and duration of corticosteroid therapy or whether treatment alters the course of the disease, corticosteroids can be life saving in patients whom crucial organs are effected (1)
- a systemic review of eight randomized placebo controlled trials of oral and inhaled corticosteroids in patients with pulmonary disease concluded that:
- oral steroids resulted in improved appearance on chest radiography and a global score of such appearances, symptoms and spirometry over 3 to 24 months
- there is little evidence that there is an improvement in lung function
- there is limited data on the effect of oral steroids on long term disease progression (2)
- severe nonpulmonary sarcoidosis which includes sight threatening ocular, cardiac and neurological involvement and severe hypercalcaemia (usually >3 mmol/l) is generally treated with systemic corticosteroids (1)
- however long term benefits of corticosteroids with regard to the outcome (e.g. - prevention of lung fibrosis and/or loss of functional capacity) have not been proven yet (2)
- taking into account the potential severe side effects, a corticosteroid sparing agent could be considered at an early phase of the therapy (2)
Reference: