Table 3. Treatment recommendations for systemic manifestations of primary Sjögren’s syndrome (modified according to [30]).

Indication	Treatment (Grade of Recommendation)
Parotid swelling	– NSAIDs and short-term oral corticosteroids (<20 mg/d for max. 1 month) (d) → consideration of contraindications – Antibiotic treatment, if required (D)
Arthritis	– Hydroxychloroquine (C) → Caution: annual ophthalmological follow-ups – NSAIDs, short-term oral/intraarticular corticosteroids (C); → consideration of contraindications – Second-line DMARDs as with rheumatoid arthritis (C), (especially methotrexate [D] → Caution: renal failure)
Interstitial pneumopathy	– Corticosteroids, oral or intravenous (C) → consideration of contraindications – Cyclophosphamide for active alveolitis (C) → Caution: hemorrhagic cystitis, pancytopenia, fertility – Pirfenidone, Nintedanib (C) → Caution: hepatic impairment
Tubulointerstitial nephritis	– Potassium and bicarbonate replacement (D) → 3 × 1–2 g/day
Peripheral neuropathy	– Antidepressants, gabapentin (D) → Caution: sicca – Corticosteroids, oral or intravenous, IVIg (D) → consideration of contraindications
Cryoglobulinemic vasculitis with organ involvement	– Methylprednisolone, plasmapheresis (C) → consideration of contraindications – Rituximab (C) → Caution: infusion reaction, formation of immune complexes, infections
B-cell lymphoma	– Treatment protocols according to subentity and stage

Close monitoring of patients treated with DMARDs or immunosuppressants according to the recommendations of the German Society of Rheumatology (dgrh.de/therapieueberwachen.html)Grades of Recommendation according to Centre for Evidence-based Medicine in Oxford:

(A) Evidence obtained from meta-analyses or at least one randomized controlled trial;

(B) Evidence from at least one well-designed experimental study;

(C) Evidence from at least one well-designed descriptive study or case–control studies;

(D) Evidence from expert opinion;

DMARDs, disease-modifying antirheumatic drugs;

IVIg, intravenous immunoglobulins;

NSAID, nonsteroidal anti-inflammatory drugs