|Systemic Sclerosis (Scleroderma)
Inflammation and fibrosis of exocrine glands with Dry eyes, dry mouth and dry vagina and systemic symptoms
- Autoimmune destruction of lacrimal and salivary glands
- Dry eyes and dry mouth are called the sicca syndrome.
- Dry mouth xerostomia, dry eyes xerophthalmia.
- Primary Sjögren’s syndrome (PSS): Autoimmune disease seen in middle-aged older lady and 50% are RF positive
- Secondary Sjögren’s syndrome: symptoms come on with
- Rheumatoid arthritis
- SLE, Scleroderma
- Primary Biliary Cirrhosis. 95% RF positive
- Chronic Lymphocytic infiltration and fibrosis of exocrine glands
- Mainly lacrimal and salivary: dry mouth and dry eyes
- Autoimmune disease which presents with dry mouth, eyes, skin and vagina
- Association with HLA B8/DR3
- Females x 9 and onset age 30-40s
- Dry Mouth: dental caries, parotitis, bad breath
- Keratoconjunctivitis sicca: dry gritty eyes + corneal + conjunctival irritation. Positive Schirmer test
- Vaginal dryness : dyspareunia
- Arthritis/Arthralgia: which can be erosive
- Vasculitis, Raynaud's phenomenon
- Fatigue, Myalgia, Myositis, Arthralgia, Lymphadenopathy
- Vasculitis purpura, neuropathies, Pancreatitis
- Renal tubular acidosis
- Thyroid disease
- Autoimmune hepatitis
- Primary biliary cirrhosis
- Non-Hodgkin's B-cell lymphoma.
- FBC: anaemia, low WCC can be seen
- Elevated ESR/CRP reflext disease activity
- Typically rheumatoid factor is +ve in 38%.
- ANA positive in 80% cases
- Anti-Ro (SSA in 60%) & anti-La (SSB in 26%) antibodies may be present (in pregnancy, these cross the placenta and cause fetal congenital heart block in 5%)
- Polyclonal hypergammaglobulinaemia
- Schirmer's test: measures conjunctival dryness. A strip of filter paper placed on lower lid with wetting. Less than <5mm in 5min is +ve and confirms defective tear production.
- Rose Bengal score or other ocular dye score shows punctate or filamentary keratitis (use a slit-lamp).
- Labial gland biopsy can aid diagnosis. May show lymphocytic infiltration of the exocrine glands
- Treatment symptomatic with artificial tears/saliva replacement solutions
- Symptomatic: Dry mouth so drinking plenty and moistening mouth sprays
- Xerophthalmia: Artificial tears (e.g. Hypromellose), eye lubricant ointments. Ophthalmic review.
- Ciclosporin eyedrops recently approved in the US
- NSAIDS and Hydroxychloroquine for arthritis
- Systemic disease - Hydroxychloroquine, Methotrexate and steroids for systemic problems