SYSTEMIC SCLEROSIS Flashcards
A 45-year-old woman presents with episodic discoloration of her fingers, which turn white and then blue in cold weather and during emotional stress. After rewarming, her fingers turn red. She has no history of digital ulcers or other systemic symptoms. What is the most likely diagnosis?
A) Primary Raynaud’s disease
B) Secondary Raynaud’s phenomenon due to systemic sclerosis
C) Vasculitis
D) Peripheral arterial disease
A) Primary Raynaud’s disease
Rationale: This patient presents with episodic changes in color, typical of Raynaud’s phenomenon, but without any signs of underlying disease, digital ulcers, or other systemic symptoms. This suggests Primary Raynaud’s disease, which is an exaggerated vasomotor response to cold or stress without an underlying condition. In contrast, Secondary Raynaud’s phenomenon (B) would typically present with more severe symptoms, including digital ulcers or tissue necrosis, and a history of connective tissue disease.
A 55-year-old woman with systemic sclerosis (SSc) presents with worsening digital ulcers and a history of prolonged, painful episodes of finger discoloration. Which of the following diagnostic tools would most help differentiate primary from secondary Raynaud’s phenomenon?
A) Nailfold capillaroscopy
B) ANA test
C) Cold stimulation test
D) Doppler ultrasound
A) Nailfold capillaroscopy
Rationale: Nailfold capillaroscopy is a critical diagnostic tool in differentiating primary Raynaud’s disease from secondary Raynaud’s phenomenon. In secondary Raynaud’s, such as in SSc, the capillaries are typically distorted, with widened and irregular loops, dilated lumens, microhemorrhages, and areas of vascular dropout.
A 40-year-old male with Raynaud’s phenomenon is found to have a positive ANA test and is later diagnosed with systemic sclerosis (SSc). Which of the following is most likely to be seen on nailfold capillaroscopy in this patient?
A) Evenly spaced parallel vascular loops
B) Capillary loops with a narrow lumen and sharp, defined edges
C) Irregular, widened capillary loops with microhemorrhages
D) Hypervascularity and dilated capillaries
C) Irregular, widened capillary loops with microhemorrhages
Rationale: Nailfold capillaroscopy in secondary Raynaud’s phenomenon, particularly in conditions like SSc, shows irregular, widened capillary loops, microhemorrhages, and areas of vascular dropout.
What is the typical presentation of scleroderma renal crisis (SRC)?
A) Low blood pressure with normal kidney function
B) Gradual, asymptomatic renal failure over months
C) Accelerated hypertension, oliguric renal insufficiency, and microangiopathic hemolysis
D) Acute hyperkalemia with normal blood pressure
C) Accelerated hypertension, oliguric renal insufficiency, and microangiopathic hemolysis
Rationale: Scleroderma renal crisis often presents with accelerated hypertension (generally >150/90 mmHg), progressive oliguric renal insufficiency, and microangiopathic hemolysis. Some patients may present normotensive, but this is typically associated with a poor prognosis.
Which of the following antibodies is associated with an increased risk of interstitial lung disease (ILD) and poor outcomes in systemic sclerosis (SSc)?
A) Anti-centromere antibodies
B) Anti-topoisomerase I (Scl-70) antibodies
C) Anti-U3-RNP antibodies
D) Anti-RNA polymerase III antibodies
B) Anti-topoisomerase I (Scl-70) antibodies
Rationale: Anti-topoisomerase I (Scl-70) antibodies are associated with increased risk of interstitial lung disease (ILD) and poor outcomes in patients with systemic sclerosis (SSc).
Which of the following antibodies is most commonly associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc)?
A) Anti-topoisomerase I (Scl-70) antibodies
B) Anti-centromere antibodies
C) Anti-RNA polymerase III antibodies
D) Anti-nucleolar antibodies
B) Anti-centromere antibodies
Rationale: Anti-centromere antibodies are associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc), although they are less frequently associated with significant cardiac, pulmonary, or renal involvement.
