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

ONGOING

dry eyes

1st line – artificial tears alone

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dry eyes
1st line – 

artificial tears alone

Artificial tears used alone are first-line therapy. If they are insufficient, the subsequent therapies are indicated, but artificial tears may continue to be used when required as an adjunct to all other therapies for dry eyes.

2nd line – ophthalmic ciclosporin drops

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dry eyes
2nd line – 

ophthalmic ciclosporin drops

Patient should wait at least 15 minutes after administration of ciclosporin before using artificial tears.

Primary options

ciclosporin ophthalmic: (0.05%, 0.09%, 0.1%) 1 drop into the affected eye(s) every 12 hours

Consider – artificial tears

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dry eyes
Consider – 

artificial tears

Additional treatment recommended for SOME patients in selected patient group

Artificial tears may continue to be used as needed as an adjunct to all other therapies for dry eyes.

Consider – ophthalmic corticosteroid drops

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dry eyes
Consider – 

ophthalmic corticosteroid drops

Additional treatment recommended for SOME patients in selected patient group

Intermittent corticosteroid eye drops may be helpful as an adjunct therapy for patients being treated with ciclosporin eye drops to reduce the time to symptom relief, or for patients with ocular inflammation who have not responded to artificial tears/lubricants and topical ciclosporin eye drops.[90][91][92]

Primary options

loteprednol ophthalmic: (0.5%) 1-2 drops into the affected eye(s) four times daily

Consider – spectacle eye shields

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dry eyes
Consider – 

spectacle eye shields

Additional treatment recommended for SOME patients in selected patient group

A recommended adjunct to help maintain a humid environment. Also, patients should take regular breaks while reading.[88]

Consider – humidifiers

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dry eyes
Consider – 

humidifiers

Additional treatment recommended for SOME patients in selected patient group

Humidify to alleviate loss of secretions by evaporation.

Consider – punctal plugs or permanent punctal occlusion

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dry eyes
Consider – 

punctal plugs or permanent punctal occlusion

Additional treatment recommended for SOME patients in selected patient group

Punctal plugs or permanent punctal occlusion may be an adjunct to other treatment when artificial tears and eye drops are insufficient to provide relief for dry eyes.[87]

Significant improvements in Schirmer's test, staining with Rose Bengal/fluorescein, and tear break-up time have been described in 19 patients with primary Sjogren syndrome at 24 months, after thermal punctal occlusion.[93]

3rd line – cholinergic drug

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dry eyes
3rd line – 

cholinergic drug

Cholinergic drugs stimulate secretion by exocrine glands.

Best to avoid systemic pilocarpine for patients with respiratory disease (e.g., chronic bronchitis, asthma, and chronic obstructive pulmonary disease) and those taking antihypertensive medicines because, even though no notable drug interactions have been reported, interactions with beta-blockers appear to be possible.[114]

Primary options

pilocarpine: 5 mg orally three times daily

Secondary options

cevimeline: 30 mg orally three times daily

Consider – artificial tears

Back
ONGOING
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dry eyes
Consider – 

artificial tears

Additional treatment recommended for SOME patients in selected patient group

Artificial tears may continue to be used as needed as an adjunct to all other therapies for dry eyes.

Consider – ophthalmic corticosteroid drops

Back
ONGOING
|
dry eyes
Consider – 

ophthalmic corticosteroid drops

Additional treatment recommended for SOME patients in selected patient group

Intermittent corticosteroid eye drops may be helpful as an adjunct therapy for patients with ocular inflammation who have not responded to artificial tears/lubricants and topical ciclosporin eye drops.[90][91][92]

Primary options

loteprednol ophthalmic: (0.5%) 1-2 drops into the affected eye(s) four times daily

Consider – spectacle eye shields

Back
ONGOING
|
dry eyes
Consider – 

spectacle eye shields

Additional treatment recommended for SOME patients in selected patient group

A recommended adjunct to help maintain a humid environment. Also, patients should take regular breaks while reading.[88]

Consider – humidifiers

Back
ONGOING
|
dry eyes
Consider – 

humidifiers

Additional treatment recommended for SOME patients in selected patient group

Humidify to alleviate loss of secretions by evaporation.

Consider – punctal plugs or permanent punctal occlusion

Back
ONGOING
|
dry eyes
Consider – 

punctal plugs or permanent punctal occlusion

Additional treatment recommended for SOME patients in selected patient group

Punctal plugs may be an adjunct to other treatment when artificial tears and eye drops are insufficient to provide relief for dry eyes.[87]

Significant improvements in Schirmer's test, staining with Rose Bengal/fluorescein, and tear break-up time have been described in 19 patients with primary Sjogren syndrome at 24 months, after thermal punctal occlusion.[93]

dry mouth

1st line – salivary substitutes alone

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dry mouth
1st line – 

salivary substitutes alone

Salivary substitutes for improving lubrication and hydration of oral tissues are used alone as first-line therapy.[36]

If they prove insufficient, they may continue to be used as needed as an adjunct to subsequent treatment options.

Gels (fluoride gels), saliva-stimulating lozenges or chewing gums, mouthwashes, prescription-strength toothpastes, and oral rinses have been used for this purpose.

Temporary comfort can be obtained by sipping water and other sugar-free liquids.

2nd line – cholinergic drug

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ONGOING
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dry mouth
2nd line – 

cholinergic drug

Cholinergic drugs stimulate secretion by exocrine glands.

Best to avoid systemic pilocarpine for patients with respiratory disease (e.g., chronic bronchitis, asthma, and chronic obstructive pulmonary disease) and those taking antihypertensive medicines because, even though no notable drug interactions have been reported, interactions with beta-blockers appear to be possible.[114]

Primary options

pilocarpine: 5 mg orally three times daily

OR

cevimeline: 30 mg orally three times daily

Consider – salivary substitutes

Back
ONGOING
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dry mouth
Consider – 

salivary substitutes

Additional treatment recommended for SOME patients in selected patient group

Salivary substitutes for improving lubrication and hydration of oral tissues are used alone as first-line therapy.[36]​

If they prove insufficient, they may continue to be used as required as an adjunct to subsequent treatment options.

Gels (fluoride gels), saliva-stimulating lozenges or chewing gums, mouthwashes, prescription-strength toothpastes, and oral rinses have been used for this purpose.

Temporary comfort can be obtained by sipping water and other sugar-free liquids.

Consider – humidifiers and moisturisers

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ONGOING
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dry mouth
Consider – 

humidifiers and moisturisers

Additional treatment recommended for SOME patients in selected patient group

Humidify and moisturise to alleviate loss of secretions by evaporation.

Moisturising creams or petroleum jelly on lips may help prevent cracking and dryness.

with fatigue

1st line – treatment of comorbid causes

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with fatigue
1st line – 

treatment of comorbid causes

Fatigue affects about 50% of patients. There is no specific treatment.

Concomitant hypothyroidism, fibromyalgia, lymphoma, or underlying depression should be considered, and treated if present. See Primary hypothyroidism, Fibromyalgia, Non-Hodgkin's lymphoma, Hodgkin's lymphoma, and Depression in adults.

A consensus recommendation suggests that hydroxychloroquine may be used to treat fatigue, but there are no randomised clinical trials supporting this intervention.[109]​​ Potential use of hydroxychloroquine should only be considered after comprehensive evaluation of disease activity, sicca manifestations, and subjective variables, and should be individualised according to the clinical context.[109]​​

Primary options

hydroxychloroquine: consult specialist for guidance on dose

with musculoskeletal manifestations

1st line – analgesia

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with musculoskeletal manifestations
1st line – 

analgesia

If effective, simple analgesics such as paracetamol are a safer alternative to non-steroidal anti-inflammatory drugs (NSAIDs) and should be used in preference.

If simple analgesics are ineffective or contraindicated, then NSAIDs can be used, although the use of NSAIDs for arthralgia/myalgia/arthritis in patients with Sjogren syndrome is not evidence-based. In older patients, it is recommended to avoid using NSAIDs at high doses due to risk of gastropathy.

Primary options

paracetamol: 500-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day

Secondary options

ibuprofen: 300-800 mg orally three to four times daily when required, maximum 3200 mg/day

OR

naproxen: 250-500 mg orally twice daily when required, maximum 1500 mg/day

OR

diclofenac potassium: 50 mg orally (immediate-release) three to four times daily when required, maximum 200 mg/day

2nd line – disease-modifying anti-rheumatic drug

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with musculoskeletal manifestations
2nd line – 

disease-modifying anti-rheumatic drug

Hydroxychloroquine is recommended as a first-line disease-modifying anti-rheumatic drug (DMARD) for inflammatory musculoskeletal pain in patients with primary Sjogren syndrome.[109]​​

If the patient does not respond to hydroxychloroquine alone, they should be switched to methotrexate alone.[109]​

If both monotherapies are ineffective, combination treatment with hydroxychloroquine plus methotrexate can be considered.[109]​

Primary options

hydroxychloroquine: consult specialist for guidance on dose

Secondary options

methotrexate: consult specialist for guidance on dose

Tertiary options

hydroxychloroquine: consult specialist for guidance on dose

and

methotrexate: consult specialist for guidance on dose

Consider – analgesia

Back
ONGOING
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with musculoskeletal manifestations
Consider – 

analgesia

Additional treatment recommended for SOME patients in selected patient group

If effective, simple analgesics such as paracetamol are a safer alternative to non-steroidal anti-inflammatory drugs (NSAIDs) and should be used in preference.

If simple analgesics are ineffective or contraindicated, then NSAIDs can be used, although the use of NSAIDs for arthralgia/myalgia/arthritis in patients with Sjogren syndrome is not evidence-based. In older patients, it is recommended to avoid using NSAIDs at high doses due to risk of gastropathy.

Primary options

paracetamol: 500-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day

Secondary options

ibuprofen: 300-800 mg orally three to four times daily when required, maximum 3200 mg/day

OR

naproxen: 250-500 mg orally twice daily when required, maximum 1500 mg/day

OR

diclofenac potassium: 50 mg orally (immediate-release) three to four times daily when required, maximum 200 mg/day

3rd line – corticosteroid

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with musculoskeletal manifestations
3rd line – 

corticosteroid

A short course (1 month or less) of oral corticosteroids is an option for patients who do not respond to hydroxychloroquine with methotrexate.[109]​​ A longer course of corticosteroids may be effective, but a corticosteroid-sparing agent should be added as soon as possible.[109]​​

Primary options

prednisolone: 1 mg/kg orally once daily initially, then taper dose gradually in 2-3 week period

Consider – analgesia

Back
ONGOING
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with musculoskeletal manifestations
Consider – 

analgesia

Additional treatment recommended for SOME patients in selected patient group

If effective, simple analgesics such as paracetamol are a safer alternative to non-steroidal anti-inflammatory drugs (NSAIDs) and should be used in preference.

If simple analgesics are ineffective or contraindicated, then NSAIDs can be used, although the use of NSAIDs for arthralgia/myalgia/arthritis in patients with Sjogren syndrome is not evidence-based. In older patients, it is recommended to avoid using NSAIDs at high doses due to risk of gastropathy.

Primary options

paracetamol: 500-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day

Secondary options

ibuprofen: 300-800 mg orally three to four times daily when required, maximum 3200 mg/day

OR

naproxen: 250-500 mg orally twice daily when required, maximum 1500 mg/day

OR

diclofenac potassium: 50 mg orally (immediate-release) three to four times daily when required, maximum 200 mg/day

4th line – alternative immunosuppressant

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with musculoskeletal manifestations
4th line – 

alternative immunosuppressant

If the patient does not respond to initial treatments, leflunomide, sulfasalazine, azathioprine, or ciclosporin can be considered.[109] However, the recommendations for these treatments are based on weaker evidence, and choice of treatment should be guided by the physician's experience and the needs of the individual patient.[109]​

If there is major organ involvement in patients with primary Sjogren syndrome, azathioprine may be a better choice than leflunomide or sulfasalazine for the treatment of all complications including inflammatory musculoskeletal pain.[109]​

Primary options

leflunomide: consult specialist for guidance on dose

OR

sulfasalazine: consult specialist for guidance on dose

OR

azathioprine: consult specialist for guidance on dose

OR

ciclosporin: consult specialist for guidance on dose

Consider – analgesia

Back
ONGOING
|
with musculoskeletal manifestations
Consider – 

analgesia

Additional treatment recommended for SOME patients in selected patient group

If effective, simple analgesics such as paracetamol are a safer alternative to non-steroidal anti-inflammatory drugs (NSAIDs) and should be used in preference.

If simple analgesics are ineffective or contraindicated, then NSAIDs can be used, although the use of NSAIDs for arthralgia/myalgia/arthritis in patients with Sjogren syndrome is not evidence-based. In older patients, it is recommended to avoid using NSAIDs at high doses due to risk of gastropathy.

Primary options

paracetamol: 500-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day

Secondary options

ibuprofen: 300-800 mg orally three to four times daily when required, maximum 3200 mg/day

OR

naproxen: 250-500 mg orally twice daily when required, maximum 1500 mg/day

OR

diclofenac potassium: 50 mg orally (immediate-release) three to four times daily when required, maximum 200 mg/day

with vasculitis

1st line – corticosteroid

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with vasculitis
1st line – 

corticosteroid

The most common manifestation of vasculitis is skin rash, usually palpable purpura. Most patients have only a single lifetime episode of skin vasculitis. Treatment is a short course of corticosteroids.[110]

Primary options

prednisolone: 1 mg/kg orally once daily initially, then taper dose gradually in 2-3 week period

2nd line – intravenous immunoglobulin

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with vasculitis
2nd line – 

intravenous immunoglobulin

Intravenous immunoglobulin treatment in more severe/resistant cases may be effective, but therapeutic experience in vasculitis is limited.[110]

Primary options

normal immunoglobulin human: 0.4 g/kg/day intravenously for 5 days

3rd line – rituximab

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with vasculitis
3rd line – 

rituximab

Rituximab may be considered for patients with primary Sjogren syndrome with vasculitis or cryoglobulinaemia associated with vasculitis manifestations if they have experienced unacceptable adverse effects or toxicity with corticosteroids or other agents, or they are not able to taper and discontinue corticosteroid treatment.[109]​​

Primary options

rituximab: consult specialist for guidance on dose

with renal tubular acidosis

1st line – potassium repletion and alkali

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with renal tubular acidosis
1st line – 

potassium repletion and alkali

A few patients may have severe manifestations of proximal renal tubular acidosis (RTA), even presenting with profound hypokalaemia. The usual therapy of type 2 RTA of any aetiology should be instituted. This includes potassium repletion and alkali by mouth. See Renal tubular acidosis.

with neuropathy

1st line – intravenous immunoglobulin

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with neuropathy
1st line – 

intravenous immunoglobulin

A substantial minority of patients have a peripheral sensory neuropathy. Occasionally the neuropathy is severe such that gait is impaired. There are several case reports and small case series in which intravenous immunoglobulin has been effective.[111][112][113]

Primary options

normal immunoglobulin human: 0.4 g/kg/day intravenously for 5 days

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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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