Table 3.
Treatment of Sjögren’s syndrome (adapted from [1])
| Clinical Manifestation | Treatment | |
|---|---|---|
| Sicca symptoms | Xerophthalmia |
1) ED (≥ 2 x per day) and eye gels/ointments 2) Topical GC/NSAID 3) CyA AT 4) Serum tear drops 4) Pilocarpine or silicone plugs in the tear duct, scleral lenses |
| Xerostomia |
1) Topical fluorides/sugar‐free chewing gum/boiled sweets/artificial aliva (mouth sprays, gels, or rinsing solutions) 2) Pilocarpine |
|
| Xerosis cutis |
1) Moisturizers 2) Topical GC |
|
| Mucous membranes |
1) Rhinitis sicca: Nose oil 2) Tracheobronchitis sicca: Pilocarpine, bromhexine, inhalation with sodium chloride 3) Dyspareunia: estrogen‐containing suppositories |
|
| Immunosuppressants and rituximab are generally not recommended due to their potential side effects. | ||
| Parotitis | Acute |
NOTE: Exclude infection! 1) Symptomatic treatment 2) GC 3) RTX/BLM |
| Chronic |
NOTE: Exclude lymphoma or other causes! ± surgery |
|
| Joints | Arthralgia |
1) NSAID 2) HCQ |
| Arthritis |
1) NSAID + HCQ 2) HCQ + GC 3) SsI 4) RTX or ABA |
|
| Skin | Sunscreen! | |
| Cutaneous LE |
1) Topical GC or HCQ +/‐ GC 2) other antimalarials +/‐ GC 3) Retinoids, SsI |
|
| Cutaneous vasculitis |
1) GC 2) Oral SsI or RTX 3) CyC ± Pex |
|
| Fatigue |
1) Exercise, endurance training 2) HCQ |
|
| Raynaud symptoms |
Keeping warm, paraffin hand baths, Ca antagonists, AT‐II receptor blockers, phosphodiesterase inhibitors Statins ± aspirin |
|
| Kidney | Tubular |
1) symptomatic (Bicarbonate or electrolyte supplementation) 2) GC 3) SsI |
| Glomerulonephritis |
1) GC 2) RTX or CyC 3) Pex |
|
| Lung | Bronchitis | Inhalation treatment (ß2 mimetics, steroids) |
| ILD |
1) GC 2) SsI 3) CyC or RTX 4) Nintedanib |
|
| PNS | Mononeuritis multiplex |
1) GC 2) oral SsI or RTX 3) CyC ± Pex |
| Axonal PN |
1) Symptomatic 2) IVIG 3) Pulses MP 4) CyC |
|
| Ganglionopathy/CIDP |
1) IVIG 2) Pulses MP 3) CyC |
|
| CNS | CNS vasculitis/NMOSD |
1) GC 2) CyC 3) RTX ± Pex, eculimumab |
| Lymphocytic meningitis |
1) Symptomatic 2) GC 3) CyC 4) RTX ± Pex, eculimumab |
|
| Symptoms resembling multiple sclerosis | Treatment for multiple sclerosis | |
| Hematological Manifestation | Neutropenia < 500 |
1) consider G‐CSF 2) GC |
| Thrombocytopenia < 20,000 | GC | |
| Hemolytic anemia |
1) GC + IVIG 2) RTX 3) Pex or CyC |
|
| NOTE: if blood count values show persistent deviation from the normal range, lymphoma or other causes must be excluded. | ||
| B cell lymphoma | Depending on the type of lymphoma (surgery, radiotherapy, chemotherapy, RTX, R‐CHOP) | |
Abbr.: ABA, Abatacept; BLM, belimumab; CIDP, chronic‐inflammatory demyelinizing polyneuropathy; CNS, central nervous system; CyA, ciclosporin; CyC, cyclophosphamide pulsed therapy; ED, eye drops; GC, glucocorticoids; HCQ, hydroxychloroquine; ILD, interstitial lung disease; IVIG, intravenous immunoglobulins; LE, lupus erythematosus; NMOSD, Neuromyelitis optica spectrum diseases; NSAID, non‐steroidal anti‐inflammatory drugs; PEX, plasma exchange; PNS, peripheral nervous system; RTX, rituximab; SjS, Sjögren’s syndrome; SsI, steroid‐sparing immunosuppressants (MTX, methotrexate; AZA, azathioprine; MMF, mycophenolate mofetil).