Connective Tissue Disease - Others

Question 1

What is Sjogren’s Syndrome?

Answer 1

Autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces

Question 2

What are the causes of Sjogren’s Syndrome

Answer 2

Lymphocyte mediated autoimmune destruction (type 4 hypersensitivity reaction) of minor salivary and lacrimal glands which can lead to atrophy and fibrosis

Question 3

What are the symptoms of Sjogren’s syndrome?

Answer 3

• Reduced tear secretion (keratoconjunctivitis sicca) which causes dry, gritty eyes.
• Dryness of mouth (xerostomia) which can cause difficulty swallowing food.
• Vaginal dryness, reduced GI secretion

Question 4

What are the extraglandular features of sjogrens?

Answer 4

• Systemic upset,
• Arthritis,
• Raynaud’s
• Cutaneous vasculitis,
• Increased risk of lymphoma

Question 5

What are the investigations for Sjogren’s Syndrome?

Answer 5

Antibodies: Anti-Ro, Anti-La,
Inflammatory markers
Schirmer’s test (shows reduced tear production)
Salivary gland flow rate
Salivary gland biopsy

Question 6

What is the management of Sjogren’s syndrome?

Answer 6

Artificial tears and saliva.
Arthritis can be treated with hydroxychloroquine and NSAIDs

Question 7

What is the management of Sjogren’s syndrome?

Answer 7

Artificial tears and saliva.
Arthritis can be treated with hydroxychloroquine and NSAIDs

Question 8

What are the complication’s of Sjogren’s?

Answer 8

• Occular infections,
• Oral problems (cavities and candidia)
• Vaginal problems
• Other organ involvement may result in complications such as pneumonia, non-hodgkins’s lymphoma

Question 9

What is systemic sclerosis?

Answer 9

Autoimmune inflammatory condition and a fibrotic connective tissue disease. There are two main types: Limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis

Question 10

Describe features of limited cutaneous systemic sclerosis

Answer 10

Used to be called CREST syndrome:
Calcinosis (calcium deposits under the skin),
Raynaud’s phenomenon (may be the first sign),
oEsophageal dysmotility,
Sclerodactyly (skin tightens around joints and restricts movement),
Telangiectasia (small dilated blood vessels),

Question 11

What are the investigations for limited cutaneous systemic sclerosis?

Answer 11

Anti-centromere antibodies

Question 12

What are the features of diffuse cutaneous systemic sclerosis?

Answer 12

It presents with the CREST symptoms as well as cardiovascular problems, lung problems and renal problems

Question 13

What are the cardiovascular, respiratory, GI and renal features of diffuse cutaneous systemic sclerosis?

Answer 13

CV - Raynaud’s, pericarditis , myocardial fibrosis.
Resp - Pulmonary fibrosis, pulmonary hypertension
GI - Oesophageal dysmotility, bowel hypomotility
Renal - Scleroderma renal crisis which presents with hypertension.

Question 14

What antibodies can be seen in diffuse cutaneous systemic sclerosis?

Answer 14

• ANA positive (sensitive but not specific),
• Anti-Scl-70 antibodies

Question 15

What is the management of systemic sclerosis?

Answer 15

• Steroids and immunosuppressants however there is no standardised treatment.
• Nifedipine for Raynaud’s.
• PPIs and pro-motility meds for GI
• Antihypertensive meds
• Non-medical management: Avoid smoking, gentle skin stretching, regular emollients, physio

Question 16

What is poly/dermato-myositis?

Answer 16

Inflammatory disorder causing symmetrical, proximal muscle weakness. It can be associated with malignancy and can manifest as a paraneoplastic syndrome.

Question 17

What is the presentation of polymyositis?

Answer 17

Bilateral, proximal muscle weakness.
Respiratory muscle weakness
Pharyngeal muscle weakness leasing to dysphonia and dysphagia
Interstitial lung disease

Question 18

What is the presentation of dermatomyositis?

Answer 18

Same muscular featurs as polymyositis as well as:
- Heliotrope rash,
- Gottron’s papules,
- Macular erythematous rash,
- Calcinosis,
- Cutaneous vasculitis
- Nailfold erythema

Question 19

What are some extra-muscular features of polymyositis and dermatomyositis?

Answer 19

• Systemic upset (tiredness, fever, malaise, weight loss)
• Polyarthralgia
• ILD
• Reynaud’s
• Myocarditis

Question 20

What are the investigations for poly/dermato-myositis?

Answer 20

• Creatinine Kinase,
• EMG (electromyography). This is used to detect neuromuscular abnormalities
• Muscle biopsy (needed for definitive diagnosis)
• Anti-jo-1 antibodies (poly) and Anti-Mi-1 antibodies (dermato)
• Bloods: Elevated CK, LDH, AST, ALT, Aldolase

Question 21

What is the treatment for poly/dermato-myositis?

Answer 21

Steroids.
May also need other immunosuppresants such as methotrexate or azathioprine

Question 22

What is antisynthestase syndrome?

Answer 22

• Autoantibodies against aminoacyl-tRNA synthetase eg, anti-jo1.
• Myositis, ILD, thickened cracked skin on hands, raynaud’s.