Sjogren syndrome

Ans. is ‘b’ i.e., Lymphocytes

o The earliest histological finding in both the major and minor salivary glands is periductal and perivascular lymphocytic infilteration which eventually becomes extensive

Ans. is ‘a’ i.e., Parotid glands 

Answer is C (Associated with Rheumatoid Arthritis):

Kelly’s Rheumatoid arthritis is associated with Secondary Sjogren’s Syndrome and not Primary Sjogren’s Syndrome.

Keratoconjunctivitis Sicca (Sensation of sand or gravel in eyes) and salivary gland enlargement are typical symptoms of Sjogren’s syndrome (Both Primary and Secondary). Primary Sjogren’s syndrome typically affects women in their middle age (Female to male ratio = 9:1) but it may occur at any age including childhood. Primary Sjogren’s syndrome

has been reported infrequently in children with onset as early as 5 years of age_. The presence of symptoms and signs of Sjogren’s ‘s syndrome (Dry eyes; Dry mouth; Salivary Gland Enlargement) in the setting of another connective tissue disease or chronic inflammatory pathology like Rheumatoid Arthritis, SLE, Systemic Sclerosis by definition is termed as Secondary Sjogren’s Syndrome.

Ans. In primary Sjogren’s syndrome, kerato­conjunctivitis sicca is associated with rheumatoid arthritis

Ans. C: Epiphora

SjOgren’s syndrome can exist as a disorder in its own right (Primary Sjogren’s syndrome) or it may develop years after the onset of an associated rheumatic disorder such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, primary biliary cirrhosis etc. (Secondary SjOgren’s syndrome)

Ans. B: Males are commonly affected

SjOgren’s Syndrome (SS) is a systemic autoimmune disease characterised by lymphocytic infiltration, acinar cell destruction and proliferation of duct epithelium in all salivary and larimal gland tissue.

Extra glandular involvement of muscles, blood vessels, lungs, kidneys may also occur.

Females are affected more than the males

It may be primary and secondary (when associated with other connective tissue diseases). There is a risk of progression to lymphoid malignancy.

Dry mouth, dry eyes and arthritis/arthralgia are the 3 common presenting features.

Of the extraarticular manifestations, Raynaud’s phenomena is the most common skin manifestation seen in 35% of patients. Vasculitis has been reported in 5% of patients with Sjogren’s syndrome. This includes small vessel leucocytoclastic vasculitis and medium vessel necrotising vasculitis.

Distal RTA may be silent or lead to renal stones, nephrocalcinosis and compromised renal function. Hypergammaglobulinemia may be due to polyclonal activation of B cells.

The diagnosis is base on the history as no single laboratory investigation is pathognomic of either primary or secondary Sjogren’s syndrome

Ans. is ‘d’ i.e., High C4 complement levels

• Lymphoa is a well-known complication of Sjogren’s syndrome Most lymphomas are extra-nodal, low grade marginal B cell lymphomas.
• Development of Lymphoma in Sjogren’s syndrome is suggested by low C4 complement levels.

Lymphoma in Sjogren’s syndrome

The development ofLymphomas in patients with Sjogren syndrome is suggested by : –

• Persistent parotid gland enlargement
• Purpura
• Leukopenia
• Cryoglobulinemia
• Low C₄ complement levels