Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute respiratory failure unable to tolerate oral intake

1st line – intravenous corticosteroid

Back
ACUTE
|
acute respiratory failure unable to tolerate oral intake
1st line – 

intravenous corticosteroid

Extremely rare. Patients requiring mechanical ventilation or >40% supplemental oxygen require intravenous corticosteroid until oral intake is possible. Usually the switch to oral corticosteroids is done within 48 hours if the patient is improving and can tolerate oral intake. Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Primary options

methylprednisolone sodium succinate: 40 mg intravenously every 6 hours until oral intake possible

Plus – ventilatory support and oxygen

Back
ACUTE
|
acute respiratory failure unable to tolerate oral intake
Plus – 

ventilatory support and oxygen

Treatment recommended for ALL patients in selected patient group

For patients with resting or exercise oxygen saturation ≤88% or PaO₂ ≤55 mmHg on arterial blood gas measurement.

Recommended flow rate is 2-4 L/minute, evidenced by oxygen testing to achieve a saturation of 90%.

acute respiratory failure able to tolerate oral intake

1st line – oral corticosteroid

Back
ACUTE
|
acute respiratory failure able to tolerate oral intake
1st line – 

oral corticosteroid

Extremely rare. Patients requiring mechanical ventilation or >40% supplemental oxygen require intravenous corticosteroid until oral intake is possible. Usually the switch to oral corticosteroids is done within 48 hours if the patient is improving and can tolerate oral intake.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Primary options

prednisone: 40 mg orally once daily, then taper gradually

Plus – ventilatory support and oxygen

Back
ACUTE
|
acute respiratory failure able to tolerate oral intake
Plus – 

ventilatory support and oxygen

Treatment recommended for ALL patients in selected patient group

For patients with resting or exercise oxygen saturation ≤88% or PaO₂ ≤55 mmHg on arterial blood gas measurement.

Recommended flow rate is 2-4 L/minute, evidenced by oxygen testing to achieve a saturation of 90%.

ONGOING

pulmonary disease

1st line – observation

Back
ONGOING
|
pulmonary disease
|
stage I without progressive adenopathy
1st line – 

observation

Asymptomatic patients do not require treatment but will need observation for onset of symptoms.

1st line – oral or inhaled corticosteroid

Back
ONGOING
|
pulmonary disease
|
symptomatic stage I with progressive adenopathy
1st line – 

oral or inhaled corticosteroid

Oral corticosteroids can be used for symptomatic stage I with adenopathy. Inhaled corticosteroids can also be used, but evidence for benefit is limited.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Primary options

prednisone: 20-40 mg orally once daily for 1-3 months, then taper gradually

OR

budesonide inhaled: (180 micrograms/dose breath-actuated inhaler) 180-720 micrograms inhaled twice daily

1st line – oral or inhaled corticosteroid

Back
ONGOING
|
pulmonary disease
|
symptomatic stage II, III, or IV
1st line – 

oral or inhaled corticosteroid

Oral corticosteroids are the mainstay of treatment for symptomatic stages II to IV.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Inhaled corticosteroids may benefit some patients, but randomized controlled trials provide limited evidence for efficacy.[71][73][74]

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Primary options

prednisone: 20-40 mg orally once daily until hypoxia and other respiratory symptoms respond, then taper gradually

OR

budesonide inhaled: (180 micrograms/dose breath-actuated inhaler) 180-720 micrograms inhaled twice daily

Consider – immunosuppressive agent

Back
ONGOING
|
pulmonary disease
|
symptomatic stage II, III, or IV
Consider – 

immunosuppressive agent

Treatment recommended for SOME patients in selected patient group

Methotrexate, azathioprine, leflunomide, mycophenolate, hydroxychloroquine, or other emerging/experimental therapies can be used as corticosteroid-sparing agents in patients who do not tolerate high doses of corticosteroids or as additional treatment in cases of unsatisfactory response to corticosteroids.[14][30]​​​[68]

The choice of agent depends on the specialist's experience and individual preference.

Subspecialist referral should be considered in cases not responding to first-line treatment or when a second agent is considered.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day of each week initially, increase gradually according to response, maximum 15 mg/week

OR

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

Secondary options

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

OR

leflunomide: 10-20 mg orally once daily

More

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

Consider – oxygen

Back
ONGOING
|
pulmonary disease
|
symptomatic stage II, III, or IV
Consider – 

oxygen

Treatment recommended for SOME patients in selected patient group

For patients with resting or exercise oxygen saturation ≤88% or PaO₂ ≤55 mmHg on arterial blood gas measurement.

Recommended flow rate is 2-4 L/minute, evidenced by oxygen testing to achieve a saturation of 90%.

2nd line – immunosuppressive agent

Back
ONGOING
|
pulmonary disease
|
symptomatic stage II, III, or IV
2nd line – 

immunosuppressive agent

Options include methotrexate, azathioprine, leflunomide, mycophenolate, or hydroxychloroquine.

The choice of agent depends on the specialist's experience and individual preference.

Subspecialist referral should be considered in cases not responding to first-line treatment or when a second agent is considered.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

OR

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

Secondary options

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

OR

leflunomide: 10-20 mg orally once daily

More

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

Consider – oxygen

Back
ONGOING
|
pulmonary disease
|
symptomatic stage II, III, or IV
Consider – 

oxygen

Treatment recommended for SOME patients in selected patient group

For patients with resting or exercise oxygen saturation ≤88% or PaO₂ ≤55 mmHg on arterial blood gas measurement.

Recommended flow rate is 2-4 L/minute, evidenced by oxygen testing to achieve a saturation of 90%.

3rd line – biologic agent

Back
ONGOING
|
pulmonary disease
|
symptomatic stage II, III, or IV
3rd line – 

biologic agent

Biologic agents, typically infliximab, should be considered if second-line agents (alone or in combination with corticosteroids) are toxic or insufficiently effective or if severe or progressive disease is present.[68][76]

Primary options

infliximab: consult specialist for guidance on dose

Consider – oxygen

Back
ONGOING
|
pulmonary disease
|
symptomatic stage II, III, or IV
Consider – 

oxygen

Treatment recommended for SOME patients in selected patient group

For patients with resting or exercise oxygen saturation ≤88% or PaO₂ ≤55 mmHg on arterial blood gas measurement.

Recommended flow rate is 2-4 L/minute, evidenced by oxygen testing to achieve a saturation of 90%.

1st line – lung transplant

Back
ONGOING
|
pulmonary disease
|
end-stage lung disease
1st line – 

lung transplant

Patients are said to have end-stage lung disease when there are severe reductions in lung volumes, flow rates, and diffusing capacity of the lung for carbon monoxide, accompanied by severe symptoms that have exhausted all available treatments. Lung transplant, if desired, is the only therapeutic option for these patients. Sarcoidosis is the second most common indication of lung transplant for interstitial lung disease and represents 2.6% of all lung transplants.[82]

cutaneous disease

1st line – topical corticosteroid

Back
ONGOING
|
cutaneous disease
|
erythematous scaly rash and/or papules
1st line – 

topical corticosteroid

Topical corticosteroids are the initial treatment for erythematous scaly lesions. Potent topical corticosteroids are contraindicated on the face.

Primary options

triamcinolone topical: (0.1%) apply to the affected area(s) two to four times daily

Consider – intralesional corticosteroid

Back
ONGOING
|
cutaneous disease
|
erythematous scaly rash and/or papules
Consider – 

intralesional corticosteroid

Treatment recommended for SOME patients in selected patient group

Intralesional corticosteroids may also be used for prominent papular or nodular lesions.

Typically more effective with injections repeated at 2- to 3-week intervals.[83]

Primary options

triamcinolone acetonide: 1-3 mg intralesionally, maximum 30 mg/lesion, repeat if necessary after 2-3 weeks

2nd line – oral corticosteroid

Back
ONGOING
|
cutaneous disease
|
erythematous scaly rash and/or papules
2nd line – 

oral corticosteroid

Used for large disfiguring lesions or lesions refractory to local corticosteroids.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Primary options

prednisone: 40 mg orally once daily, then taper gradually

Consider – immunosuppressive agent

Back
ONGOING
|
cutaneous disease
|
erythematous scaly rash and/or papules
Consider – 

immunosuppressive agent

Treatment recommended for SOME patients in selected patient group

Corticosteroid-sparing agents (e.g., hydroxychloroquine, methotrexate) should be considered whenever possible.​[68][84]​​​

The choice of agent depends on the specialist's experience and individual preference.

Primary options

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

OR

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

3rd line – immunosuppressive agent

Back
ONGOING
|
cutaneous disease
|
erythematous scaly rash and/or papules
3rd line – 

immunosuppressive agent

Hydroxychloroquine or methotrexate may be used in cases refractory to corticosteroids.​[68][84]​​​

The choice of agent depends on the specialist's experience and individual preference.

Primary options

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

OR

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

4th line – biologic agent

Back
ONGOING
|
cutaneous disease
|
erythematous scaly rash and/or papules
4th line – 

biologic agent

Biologic agents, typically infliximab, are generally reserved for later use.[68][77]

Primary options

infliximab: consult specialist for guidance on dose

1st line – oral corticosteroid

Back
ONGOING
|
cutaneous disease
|
lupus pernio with scarring
1st line – 

oral corticosteroid

Indurated plaques with discoloration of the nose, cheeks, lips, and ears.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Primary options

prednisone: 40 mg orally once daily, then taper gradually

Consider – immunosuppressive agent

Back
ONGOING
|
cutaneous disease
|
lupus pernio with scarring
Consider – 

immunosuppressive agent

Treatment recommended for SOME patients in selected patient group

Methotrexate or azathioprine may be used as corticosteroid-sparing agents in people who do not respond to or do not tolerate oral corticosteroids. The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

OR

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

2nd line – immunosuppressive agent

Back
ONGOING
|
cutaneous disease
|
lupus pernio with scarring
2nd line – 

immunosuppressive agent

Methotrexate or azathioprine may be used in people who do not respond to or do not tolerate oral corticosteroids.

The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

OR

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

3rd line – biologic agent

Back
ONGOING
|
cutaneous disease
|
lupus pernio with scarring
3rd line – 

biologic agent

Biologic agents, typically infliximab, are generally reserved for later use.[68] Subgroup analyses from a clinical trial and observational data suggest that infliximab therapy may be of considerable benefit in patients with lupus pernio.[78][79]

Primary options

infliximab: consult specialist for guidance on dose

ocular disease

1st line – topical corticosteroid

Back
ONGOING
|
ocular disease
|
anterior uveitis
1st line – 

topical corticosteroid

Topical corticosteroids are the mainstay and usually are very effective; rarely need to progress to oral therapy.[41]

Primary options

prednisolone acetate ophthalmic: (1%) 1 drop into the affected eye(s) four times daily

1st line – oral corticosteroid

Back
ONGOING
|
ocular disease
|
optic neuritis and uveitis resistant to topical agents
1st line – 

oral corticosteroid

Optic neuritis and failed topical treatment are the main indications for oral corticosteroids.[41]

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Primary options

prednisone: 0.5 to 1 mg/kg orally once daily initially, then taper gradually

cardiovascular disease

1st line – oral corticosteroid

Back
ONGOING
|
cardiovascular disease
1st line – 

oral corticosteroid

A patient with cardiac involvement will usually present with arrhythmias or heart block; pulmonary hypertension and congestive heart failure occur later in the course of the condition. Refer these patients early to a cardiologist for appropriate management. A multidisciplinary team approach is required to address complex clinical issues that may arise.[38]

Higher-dose corticosteroids may be needed.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.[38]

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Primary options

prednisone: 40 mg orally once daily, followed by a gradual reduction to a maintenance dose of 5-10 mg once daily or on alternate days

Consider – immunosuppressive agent

Back
ONGOING
|
cardiovascular disease
Consider – 

immunosuppressive agent

Treatment recommended for SOME patients in selected patient group

Methotrexate, azathioprine, mycophenolate, or leflunomide may be used as corticosteroid-sparing agents.​[14][38]​​[68]​​​​

The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

OR

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

Secondary options

leflunomide: 10-20 mg orally once daily

More

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

2nd line – immunosuppressive agent

Back
ONGOING
|
cardiovascular disease
2nd line – 

immunosuppressive agent

Methotrexate, azathioprine, mycophenolate, or leflunomide may be used in refractory cases.​[14][38]​​[68]​​

The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

OR

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

Secondary options

leflunomide: 10-20 mg orally once daily

More

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

3rd line – biologic agent

Back
ONGOING
|
cardiovascular disease
3rd line – 

biologic agent

Biologic agents, typically infliximab, are generally reserved for later use.[38][68]​​​​ Infliximab has been shown to benefit patients with refractory cardiac sarcoidosis, although tumor necrosis factor (TNF)-alpha inhibitors should be used with caution because of the potential for cardiotoxicity.[38][80]​​

Primary options

infliximab: consult specialist for guidance on dose

central nervous system or peripheral nervous system involvement

1st line – oral corticosteroid

Back
ONGOING
|
central nervous system or peripheral nervous system involvement
1st line – 

oral corticosteroid

For cranial neuropathy, persistent peripheral neuropathy, and intracranial lesions.[85]

Patients with neurosarcoidosis invariably require corticosteroids; high doses may be prescribed for central nervous system involvement.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Primary options

prednisone: 40 mg orally once daily, followed by a gradual reduction to a maintenance dose of 5-10 mg once daily or on alternate days

Consider – immunosuppressive agent

Back
ONGOING
|
central nervous system or peripheral nervous system involvement
Consider – 

immunosuppressive agent

Treatment recommended for SOME patients in selected patient group

Methotrexate is typically used as a corticosteroid-sparing agent in patients who do not tolerate high doses of corticosteroids or as additional treatment in cases of unsatisfactory response to corticosteroids. Other options include azathioprine, mycophenolate, and hydroxychloroquine.[14][68]

The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

Secondary options

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

OR

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

2nd line – immunosuppressive agent

Back
ONGOING
|
central nervous system or peripheral nervous system involvement
2nd line – 

immunosuppressive agent

Methotrexate is typically used in refractory cases. Other options include azathioprine, mycophenolate, and hydroxychloroquine.[14][68]

The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

methotrexate: 7.5 mg orally/subcutaneously/intramuscularly once weekly on the same day each week initially, increase gradually according to response, maximum 15 mg/week

Secondary options

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

OR

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

3rd line – biologic agent

Back
ONGOING
|
central nervous system or peripheral nervous system involvement
3rd line – 

biologic agent

Biologics, typically infliximab, are generally reserved for later use.[68]

Primary options

infliximab: consult specialist for guidance on dose

renal disease

1st line – oral corticosteroid

Back
ONGOING
|
renal disease
|
hypercalcemia/hypercalciuria
1st line – 

oral corticosteroid

Renal involvement is rare. When it occurs, it is associated with substantial risk of renal failure.[6]​ Corticosteroids are the cornerstone of treatment.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Normalization of calcium levels is expected within 1 week after treatment.

Primary options

prednisone: 20-40 mg orally once daily, followed by a gradual reduction to a maintenance dose of 5-10 mg once daily or on alternate days

Consider – hydroxychloroquine or ketoconazole

Back
ONGOING
|
renal disease
|
hypercalcemia/hypercalciuria
Consider – 

hydroxychloroquine or ketoconazole

Treatment recommended for SOME patients in selected patient group

Hydroxychloroquine or ketoconazole may be used as corticosteroid-sparing agents.[14][81]​ The choice of agent depends on the specialist's experience and individual preference.

Primary options

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

OR

ketoconazole: consult specialist for guidance on dose

2nd line – hydroxychloroquine or ketoconazole

Back
ONGOING
|
renal disease
|
hypercalcemia/hypercalciuria
2nd line – 

hydroxychloroquine or ketoconazole

Hydroxychloroquine or ketoconazole may be used in refractory cases.[14][81]​ The choice of agent depends on the specialist's experience and individual preference.

Primary options

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

OR

ketoconazole: consult specialist for guidance on dose

1st line – oral corticosteroid

Back
ONGOING
|
renal disease
|
granulomatous interstitial nephritis
1st line – 

oral corticosteroid

Renal involvement is rare. When it occurs, it is associated with substantial risk of renal failure.[6]​ Corticosteroids are the cornerstone of treatment.

Dosing and route of administration of corticosteroid therapy remain uncertain and vary depending upon the clinical setting.

Measures to prevent osteoporosis should be implemented for patients starting corticosteroids. Special care must be taken if vitamin D or calcium is supplemented in patients with sarcoidosis, because the disease may cause hypercalciuria and hypercalcemia by increased endogenous vitamin D. Serum calcium and 24-hour urinary calcium should be monitored before and 1 month after commencing therapy.[75]​ US guidelines suggest measuring both 25- and 1,25-OH vitamin D levels before vitamin D replacement therapy is started.[31]

Primary options

prednisone: 20-40 mg orally once daily, followed by a gradual reduction to a maintenance dose of 5-10 mg once daily or on alternate days

Consider – immunosuppressive agent

Back
ONGOING
|
renal disease
|
granulomatous interstitial nephritis
Consider – 

immunosuppressive agent

Treatment recommended for SOME patients in selected patient group

Azathioprine or mycophenolate may be used as corticosteroid-sparing agents.[14]​ The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

2nd line – immunosuppressive agent

Back
ONGOING
|
renal disease
|
granulomatous interstitial nephritis
2nd line – 

immunosuppressive agent

Azathioprine or mycophenolate may be used in refractory cases.[14]​ The choice of agent depends on the specialist's experience and individual preference.

Thiopurine methyl transferase (TPMT) gene mutation assays or TPMT phenotypic assays are suggested before starting therapy with azathioprine.

Primary options

azathioprine: 1 to 2.5 mg/kg/day orally given in 1-2 divided doses

OR

mycophenolate mofetil: 1 to 1.5 g orally twice daily

3rd line – biologic agent

Back
ONGOING
|
renal disease
|
granulomatous interstitial nephritis
3rd line – 

biologic agent

Biologic agents, typically infliximab, are generally reserved for later use.[6][81]

Primary options

infliximab: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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