Table 6.

General recommendations for an adequate description of systemic involvement in primary SS (articular, cutaneous, pulmonary and renal involvements)

Definition of organ-specific systemic involvement

The organ involvement should be clearly defined together with the main symptoms and the results of diagnostic tests.

The inclusion of asymptomatic patients with altered diagnostic tests in pulmonary and renal involvements should be clearly stated.

Detailing clinical presentation

For systemic presentations reported very infrequently in primary SS, we recommend to search for other associated systemic diseases, especially in patients with:

Erosive arthritis

Infrequent location of arthritis

Extracutaneous non-cryoglobulinaemic vasculitis

Proximal RTA

Always use standardized, consensual international definitions for the organ involvement: erosive arthritis, vasculitis, ILD, RTA and glomerulonephritis.

Autoantibodies studies

Specific autoantibodies related to other systemic autoimmune diseases in which organ-specific involvements overlap should be provided.

Negativity should confirm primary SS in patients presenting with:

Arthritis (SLE, RA)

AE (SLE)

Cutaneous vasculitis (ANCA vasculitides)

ILD (SSc)

GN (SLE, vasculitis).

Histopathological studies

Requirement for histopathological studies is highly recommended in some SS-related involvements (extracutaneous vasculitis, GN). It does not seem to be essential for diagnosis in other types of involvement (AE, cutaneous purpura, RTA).

AE: annular erythema; RTA: renal tubular acidosis; ILD: interstitial lung disease.