A 54-year-old woman is reviewed. She was discharged from the psychiatric ward around 5 weeks ago following an admission for an acute psychotic episode. Her psychotic symptoms have settled on risperidone but unfortunately she has now developed a dry mouth and arthralgia in both hands. A number of blood tests are requested:
What is the most likely diagnosis?
Correct Answer E: Risperidone (unlike chlorpromazine) is not a common trigger of lupus. Anti-Ro is positive in around 70% of patients with Sjogren's syndrome compared to around 10% of patients with rheumatoid arthritis.
Sjogren's syndrome:
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces. It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders, where it usually develops around 10 years after the initial onset. Sjogren's syndrome is much more common in females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold).
Features:
Investigation:
Management:
Which one of the following is most likely to indicate an underlying connective tissue disorder in a patient with Raynaud's phenomenon?
Correct Answer E: Raynaud's disease (i.e. primary) presents in young women with bilateral symptoms.
A history of recurrent miscarriages could indicate systemic lupus erythematous or anti-phospholipid syndrome.
Chilblains (pernio) are itchy, painful purple swellings which occur on the fingers and toes after exposure to the cold. They are occasionally associated with underlying connective tissue disease but this is rare.
Raynaud's:
Raynaud's phenomena may be primary (Raynaud's disease) or secondary (Raynaud's phenomenon).
Raynaud's disease typically presents in young women (e.g. 30 years old) with symmetrical attacks.
Factors suggesting underlying connective tissue disease:
Secondary causes:
A 33-year-old female is admitted to the Emergency Department due to right-sided weakness. She has a past history of deep vein thrombosis following the birth of her daughter. The only other past history of note is two miscarriages. A CT head confirms an ischaemic stroke in the left middle cerebral artery territory.
What is the likely finding on echocardiography?
Correct Answer A: This is a typical MRCP question. On first sight this question appears to be pointing towards a paradoxical embolus. However, given the history of miscarriages and DVT a diagnosis of antiphospholipid syndrome is more likely.
Antiphospholipid syndrome:
Antiphospholipid syndrome is an acquired disorder characterized by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia. It may occur as a primary disorder or secondary to other conditions, most commonly systemic lupus erythematosus (SLE).
A key point for the exam is to appreciate that antiphospholipid syndrome causes a paradoxical rise in the APTT.
This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade.
Associations other than SLE:
Management - based on BCSH guidelines:
A 35-year-old woman who has severe Raynaud's disease is reviewed in clinic. Three months ago she was started on nifedipine. Unfortunately this has had a minimal effect on her symptoms and has resulted in ankle oedema.
What is the most appropriate next step in management?
Correct Answer C:
A 63-year-old man presents to the Emergency Department with a 2 day history of a painful and swollen left knee joint. Aspiration reveals positively birefringent crystals and no organisms are seen.
Which of the following conditions are not recognized causes of the underlying condition?
Correct Answer C: A low phosphate predisposes to pseudogout.
Pseudogout:
Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate in the synovium.
Risk factors:
Click to expand