Sjogren’s Syndrome - Connective Tissue Diseases

Sjogren’s Syndrome

·        Dry eyes, dry month and associated with rheumatoid arthritis

·        Epidemiology: onset 15 – 65 years, more common in women

·        Types:

o  Primary (ie no other connective tissue disease) 

o  Secondary: associated with other connective tissue diseases: Rheumatoid (50% of Sjogren‟s have RA), SLE, Scleroderma, Polymyositis, Primary biliary cirrhosis (ie autoimmune disorders), graft-verses host disease, AIDs 

·        Presentation:

o  Gritty, sore eyes: keratoconjunctivitis sicca (¯lacrimation ® dry eyes)

o   Dry mouth: xerostomia (¯salivation) – can‟t swallow, need sips of water at night, enlarged tender parotids 

o   Also dry nose, vagina

o   Tiredness/depression

o   Arthritis as in SLE

o   Raynaud‟s

o   Pulmonary fibrosis, pleurisy

o   Also peripheral neuropathy, renal involvement, hepatosplenomegaly, pancreatitis, etc

·        Compared to RA:

o   ANA is more strongly positive in Sjogren‟s

o   Arthritis is not destructive

·        Investigations:

o   Schirmer test: < 10 mm of filter paper under the lower eye lid is wet after 5 minutes 

o   ­ESR & CRP.  May have normal CRP (can get this in most CTDs, but not RA)

o   100% have RF

o   ANA positive in 60 – 70% 

o   Anti-Ro (SSA) and Anti-La (SSB) present in 70% of primary, and 10% of secondary. NB Ro and La antibodies cross the placenta causing congenital heart block

·        Pathology:

o   Connective tissue disease 

o   Lymphocytes and plasma cells infiltrate secretory glands (also skin, lungs and liver) causing fibrosis 

o   Inflammation and destruction of exocrine glands: especially salvia and tears, with CD4+ lymphocytes

o   HLA DR3 association

·        Treatment

o   Artificial tears and saliva

o   Hydroxychloroquine and methotrexate