Table 7

 Current concepts in the extraglandular expression of primary SS

a) Cutaneous involvement
Prognostic significance of cryoglobulinaemia and cutaneous purpura4–6
Ro associated, polycyclic, photosensitive lesions (SCLE-like)4,7,8
b) Pulmonary involvement
High frequency of bronchial/bronchiolar disease (30–50%)18–20
Insidious and slow progression of pulmonary disease23
c) Renal involvement
Indolent subclinical course of TIN30
Demonstration of glomerulonephritis in 44% of patients biopsied29,30
Key role of cryoglobulinaemia in SS related glomerulonephritis29,30
d) Neurological involvement
Increased cerebral white matter lesions33
Long term insidious course and poor response to treatment of PSN48
Autonomic disturbances (abnormal responses to cardiovascular tests)51–54
e) Lymphoproliferative involvement
Prevalence of 4% of lymphoma in cross sectional studies62
Lymphadenopathy, purpura, and neuropathy as clinical markers of lymphoproliferation69
Anaemia, lymphopenia, cryoglobulins, and mIgs as analytical markers69
Salivary glands as the main site of lymphoma69
Higher prevalence of extranodal involvement69
f) Miscellaneous
Subclinical muscle inflammation53
Interstitial cystitis associated with severe urological symptoms49,50
Sensorineural hearing loss in nearly 25% of patients57–60
Rare occurrence of symptomatic cytopenias62,63,66
Autoimmune myocarditis110,111