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Clinical case: Raynaud’s syndrome: want to learn more about it?

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Clinical case: Raynaud’s syndrome

In this article we describe a case of refractive primary Raynaud's syndrome resistant to standard first-line therapy. You have probably heard about this condition, but do you truly know its sign and symptoms, pathophysiology, and diagnosis? Continue reading to find out more about Raynaud's phenomenon, its anatomy, and possible treatments.

Key facts
Raynaud's Disease It is a condition in which some of the body’s blood vessels (typically those in the fingers and toes) constrict in an exaggerated way in response to cold or emotional stress. It can be primary (idiopathic) or secondary (autoimmune).
Cervicothoracic (Stellate) ganglion It is the main relay station for the sympathetic nerve fibers traveling to the upper limb. It can be ablated or destroyed chemically for therapeutic purposes in the management of Raynaud's syndrome.
Treatments for Raynaud's syndrome

First-line: lifestyle modifications, calcium channel blockers

Alternatives: phosphodiesterase type 5 inhibitors, endothelin antagonists, prostaglandin derivatives

After reviewing this case you should be able to describe the following:

  • Raynaud’s phenomenon and the difference between primary and secondary Raynaud’s phenomenon.
  • The anatomical pathway that provides the sympathetic innervation to the upper limb. How this pathway facilitates an anatomically based treatment for Raynaud’s phenomenon.
  • Sildenafil is frequently used to treat a common condition; why did the physicians prescribe this pharmacological agent in this case?

This article is based on a case report published in the Journal "Case Reports in Surgery" in 2015, by Caroline C. Jadlowiec, Beata E. Lobel, Namita Akolkar, Michael D. Bourque, Thomas J. Devers, and David W. McFadden.

Case description

History

The patient is a 55-year-old female who was diagnosed with primary Raynaud’s phenomenon (PRP) in 2009. PRP initially displayed as a typical bilateral discoloration of the fingers varying among pallor, bluish, and reddish, accompanied by mild pain and numbness (Figure 1).

Figure 1. Typical appearance of the hand of a patient with PRP after exposure to cold temperatures (A), and then after washing in warm water (B). Note the pale distal fingers on the left hand regaining some of their normal color after the washing, and the loss of some of the redness in the palm after washing. C. shows a patient with PRP in her right foot; note the pallor of the right foot compared to the left.

The patient did not complain of joint pain, swelling, or deformity and did not show skin rashes, oral ulcers, or dysphagia. The patient’s signs and symptoms were exacerbated by cold weather and stress. She is a lifelong nonsmoker. The patient was initially treated with aspirin, nifedipine, and prednisolone at a different health facility, but no notable improvement occurred.

Physical exam

At the first exam, she did not show any features of connective tissue disease and the physical examination was remarkable because of the bilateral cyanosis of the fingertips, with a left middle finger ulcer (Figure 2A); the lower limb examination showed bluish discoloration over the distal phalanges of right and left second and third toes. Distal pulse and motor and sensory evaluations were normal.

Figure 2. A. Hands of the patient prior to the first treatment with rituximab. B. After first treatment. C. Recurrence of PRP signs. D. Disappearance of signs after second treatment.

Laboratory investigations and imaging

Laboratory blood examination for autoimmune analysis and serology, and malignancy screening were unremarkable. The patient’s condition worsened and she was admitted to the hospital with severe digital pain and ulceration. Magnetic resonance angiogram of her upper extremities did not reveal any features of vasculitis, aneurysm, or stenosis.

CT scanning of the aortic arch did not reveal any vascular abnormality. CT angiogram, however, did demonstrate attenuated, irregular right and left ulnar arteries. The patient then underwent bilateral selective ulnar angiography and distal angioplasty, the former of which indicated distal disease at the level of the palmar arches (Figure 3). Biopsy of the left little finger failed to show any significant histopathological abnormalities and was negative for vasculitis.

Figure 3. Cadaver pictures showing the superficial (A) and deep (B) palmar arches.

Treatment and evolution

Prednisolone therapy was discontinued, and the patient was given a trial of sildenafil (12.5 mg) twice a day. But this agent too failed to halt disease progression and the patient suffered from severe digital pain and bilateral ulceration of the tips of her fingers. Due to persistent symptoms unrelieved by conventional drug regimens, rituximab therapy was initiated and a significant improvement in the patient’s signs and symptoms was noted.

This treatment was continued and after six months the patient showed a complete resolution of the digital ischemia (Figure 2B). However, five years later, the patient experienced a recurrence of her previous symptoms (Figure 2C) which were resolved by a second cycle of rituximab (375 mg/m2 weekly for four weeks; Figure 2D). Currently, the patient is in complete remission.

Medical and anatomical considerations

The Raynaud phenomenon (RP) is a condition in which some of the body’s blood vessels (typically those in the fingers and toes) constrict in an exaggerated way in response to cold or emotional stress. The normal control of how blood vessels respond to cold and other stimuli is complex, involving the central nervous system, peripheral sensory nerves, and receptors within the blood vessels.

Figure 4. Cadaver image showing roots of the brachial plexus, cervicothoracic ganglion (stellate ganglion) and sympathetic trunk.

RP occurs when this complex and delicate system of control is abnormal. Classically the condition is attributed to hyperactivation of the sympathetic nervous system causing extreme vasoconstriction of the peripheral blood vessels, leading to tissue hypoxia. As shown by the current case, RP likely has a much more complex etiology than simply sympathetic system hyperactivation.

A RP episode can be triggered by exposure to cold temperature or by a shift in temperature from warm to cool. Thus, even mildly cold exposure, such as that caused by air conditioning or the cold of the refrigerated food section in a grocery store, can cause a RP attack. A general body chill can also trigger an attack, even if the hands and feet are kept warm. Emotional stress and being startled can cause an attack of RP due to the release of neurotransmitters that affect the muscular layer within the arteries.

When it comes to learning about medical conditions, the medical terminology can feel like a different language! Improve your confidence on this topic with our basic medical terminology quizzes.

The present case demonstrates the successful use of rituximab in a patient with PRP that was resistant to alternative, therapeutic management options; however, it remains unclear whether autoantibodies have a substantive role in the pathogenesis of primary or secondary RP (see Explanation 1 to Objectives). Previous studies have established the presence of antibodies against type IV collagen in patients with PRP, which may explain the response to rituximab in the present case. Rituximab is an antibody against cell surface protein CD20, which is expressed on B cells. Rituximab may induce the killing of B cells by complement-dependent cytotoxicity (CDC) or by antibody-dependent cellular cytotoxicity (ADCC), which activates natural killer cells. Further studies are required to establish the role and efficacy of this novel treatment in PRP and secondary RP.

Clinical case: Raynaud’s syndrome: want to learn more about it?

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