513226
research-article2013
AOPXXX10.1177/1060028013513226Annals of PharmacotherapyGuarneri et al
Letter
Etanercept for Psoriasis and Psoriatic Arthritis in a Patient with Charcot-MarieTooth Disease TO THE EDITOR: Charcot-Marie-Tooth (CMT) disease, also known as hereditary motor and sensory neuropathy (HMSN), encompasses a clinically and genetically heterogeneous group of disorders characterized clinically by distal muscle wasting and weakness, reduced tendon reflexes, impaired distal sensation, and variable foot deformity, and neurophysiologically by a motor and sensory neuropathy. The disease is the commonest inherited neuromuscular disorder affecting at least 1 in 2500. Charcot-Marie-Tooth disease is classified as either demyelinating (CMT1) if the median nerve motor conduction velocity is less than 38 m/s, or axonal (CMT2) if the median nerve motor conduction velocity is higher than 38 m/s. The main subtype is type 1A (CMT1A), representing about half of all CMT cases. CMT1A is associated with an autosomal dominant duplication of the peripheral myelin protein 22 gene (PMP22), causing peripheral nerve demyelination and secondary axonal loss. So far, no drug treatments are available.1,2 Tumor necrosis factor (TNF)–blocking agents have been associated with clinical deterioration in acquired demyelinating peripheral neuropathy, but our hypothesis is that patients with genetically determined demyelinating disease should not be at risk of additional worsening, in course of therapy with a TNF-blocking agent, compared with untreated patients. A 53-year-old woman developed in her 30s slight difficulty in walking and loss of feeling in her feet. She had a positive family history for CMT1A. When she was 45 years old, she was admitted to a neurologic clinic where a clinical examination and nerve conduction studies revealed a sensory-motor demyelinating polyneuropathy. She was tested for the PMP22 gene duplication that confirmed the diagnosis of CMT1A. She presented to the Dermatology Clinic of Messina University, Italy, and was hospitalized on June 2010 with an insidious onset of progressively disabling low back pain, flare-up of arthritis of the right knee, and dactyltis of the I, II, and III digits of the left foot. The patient had nail pitting and minimal psoriatic skin lesions (PASI [psoriasis area and severity index] score = 2) to the scalp, elbows, and sacral region. Erythrocyte sedimentation rate and C-reactive protein were elevated, and rheumatoid factor and anti–cyclic citrullinated peptide were negative. She was evaluated by clinical, radiological, and scintigraphic methods, and a diagnosis of psoriatic arthritis was finally established.
Annals of Pharmacotherapy 2014, Vol. 48(4) 550–551 © The Author(s) 2013 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1060028013513226 aop.sagepub.com
The patient had received a similar response from rheumatologist in the past (2006) and was treated with cyclosporine and oral prednisone alternatively for about 2 years, experiencing mild improvement of symptoms and only slight reduction in the appearance of new skin lesions. These medications were stopped in December 2008 because of high blood pressure and a “not better” defined urinary discomfort. Since then, she received oral ibuprofen 400 mg symptomatically and calcipotriol ointment, with poor control of the condition. No other systemic drugs were proposed or administered in further dermatologic and rheumatologic visits because of the underlying neurological disease. At our consultation, she was positive for hypertension and tricuspidal insufficiency, with signs of hepatic impairment. Neurological examination performed 1 week later showed bilateral pes cavus with hammer toes, slight distal wasting, and weakness in lower limbs (MRC 4 in feet dorsiflexion). She was areflexic. Sensory examination revealed reduced pinprick and vibration sense up to the ankles. Her score using Charcot-Marie-Tooth Neuropathy Score (CMTNS) was 11/36. CMTNS is a composite score based on patient symptoms, signs, and neurophysiologic abnormalities.3 A recent study about the progression of CMT1A over time, has showed that the CMTNS score increases an average of 0.686 points per year.3,4 After a preliminary diagnostic workup, including Quantiferon Gold TB testing5 and oncologic biomarkers, the patient was commenced on etanercept (25 mg twice weekly), achieving ACR 30 response and complete clearing of skin lesions after 3 months, and ACR 50 after 6 months. She was also surprised to find hair dramatically growing on the top of her head. Her scalp had been completely bald for at least 15 years, and this new hair was initially gray, then changing in distribution and finally to her original black color. She underwent clinical and neurological examination that were substantially unchanged after 6 months and after 1 year. In the latter occasion, CMTNS score was still 11. Etanercept has not been discontinued at 15 months. Both dermatological and neurological outcomes are still maintained. Biologic agents have been a significant advancement in the therapeutic management of psoriasis, with improved quality of life. Unfortunately, some of these drugs have been associated either directly or indirectly with neurological complications.
Downloaded from aop.sagepub.com at GEORGETOWN UNIV MED CTR on May 21, 2015
551
Guarneri et al Literature data still remain under debate. Treatment with TNF-blocking agents, including etanercept, has been associated with rare (
research-article2013
AOPXXX10.1177/1060028013513226Annals of PharmacotherapyGuarneri et al
Letter
Etanercept for Psoriasis and Psoriatic Arthritis in a Patient with Charcot-MarieTooth Disease TO THE EDITOR: Charcot-Marie-Tooth (CMT) disease, also known as hereditary motor and sensory neuropathy (HMSN), encompasses a clinically and genetically heterogeneous group of disorders characterized clinically by distal muscle wasting and weakness, reduced tendon reflexes, impaired distal sensation, and variable foot deformity, and neurophysiologically by a motor and sensory neuropathy. The disease is the commonest inherited neuromuscular disorder affecting at least 1 in 2500. Charcot-Marie-Tooth disease is classified as either demyelinating (CMT1) if the median nerve motor conduction velocity is less than 38 m/s, or axonal (CMT2) if the median nerve motor conduction velocity is higher than 38 m/s. The main subtype is type 1A (CMT1A), representing about half of all CMT cases. CMT1A is associated with an autosomal dominant duplication of the peripheral myelin protein 22 gene (PMP22), causing peripheral nerve demyelination and secondary axonal loss. So far, no drug treatments are available.1,2 Tumor necrosis factor (TNF)–blocking agents have been associated with clinical deterioration in acquired demyelinating peripheral neuropathy, but our hypothesis is that patients with genetically determined demyelinating disease should not be at risk of additional worsening, in course of therapy with a TNF-blocking agent, compared with untreated patients. A 53-year-old woman developed in her 30s slight difficulty in walking and loss of feeling in her feet. She had a positive family history for CMT1A. When she was 45 years old, she was admitted to a neurologic clinic where a clinical examination and nerve conduction studies revealed a sensory-motor demyelinating polyneuropathy. She was tested for the PMP22 gene duplication that confirmed the diagnosis of CMT1A. She presented to the Dermatology Clinic of Messina University, Italy, and was hospitalized on June 2010 with an insidious onset of progressively disabling low back pain, flare-up of arthritis of the right knee, and dactyltis of the I, II, and III digits of the left foot. The patient had nail pitting and minimal psoriatic skin lesions (PASI [psoriasis area and severity index] score = 2) to the scalp, elbows, and sacral region. Erythrocyte sedimentation rate and C-reactive protein were elevated, and rheumatoid factor and anti–cyclic citrullinated peptide were negative. She was evaluated by clinical, radiological, and scintigraphic methods, and a diagnosis of psoriatic arthritis was finally established.
Annals of Pharmacotherapy 2014, Vol. 48(4) 550–551 © The Author(s) 2013 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1060028013513226 aop.sagepub.com
The patient had received a similar response from rheumatologist in the past (2006) and was treated with cyclosporine and oral prednisone alternatively for about 2 years, experiencing mild improvement of symptoms and only slight reduction in the appearance of new skin lesions. These medications were stopped in December 2008 because of high blood pressure and a “not better” defined urinary discomfort. Since then, she received oral ibuprofen 400 mg symptomatically and calcipotriol ointment, with poor control of the condition. No other systemic drugs were proposed or administered in further dermatologic and rheumatologic visits because of the underlying neurological disease. At our consultation, she was positive for hypertension and tricuspidal insufficiency, with signs of hepatic impairment. Neurological examination performed 1 week later showed bilateral pes cavus with hammer toes, slight distal wasting, and weakness in lower limbs (MRC 4 in feet dorsiflexion). She was areflexic. Sensory examination revealed reduced pinprick and vibration sense up to the ankles. Her score using Charcot-Marie-Tooth Neuropathy Score (CMTNS) was 11/36. CMTNS is a composite score based on patient symptoms, signs, and neurophysiologic abnormalities.3 A recent study about the progression of CMT1A over time, has showed that the CMTNS score increases an average of 0.686 points per year.3,4 After a preliminary diagnostic workup, including Quantiferon Gold TB testing5 and oncologic biomarkers, the patient was commenced on etanercept (25 mg twice weekly), achieving ACR 30 response and complete clearing of skin lesions after 3 months, and ACR 50 after 6 months. She was also surprised to find hair dramatically growing on the top of her head. Her scalp had been completely bald for at least 15 years, and this new hair was initially gray, then changing in distribution and finally to her original black color. She underwent clinical and neurological examination that were substantially unchanged after 6 months and after 1 year. In the latter occasion, CMTNS score was still 11. Etanercept has not been discontinued at 15 months. Both dermatological and neurological outcomes are still maintained. Biologic agents have been a significant advancement in the therapeutic management of psoriasis, with improved quality of life. Unfortunately, some of these drugs have been associated either directly or indirectly with neurological complications.
Downloaded from aop.sagepub.com at GEORGETOWN UNIV MED CTR on May 21, 2015
551
Guarneri et al Literature data still remain under debate. Treatment with TNF-blocking agents, including etanercept, has been associated with rare (
