British journaJ of Dermatology (1992) 127. 300-301.
Correspondence Relapsing polychondritis—treatment with cyclosporin A SIR. The case report by Anstey et ai' was interesting and raises the question of the place of cycfosporin in the treatment of relapsing polychondritis. Because of the rarity of this disease. new information on treatment is only likely to be acquired through such case reports. We would like to add some information based on personal experience in treating a patient, and from the sparse literature on this subject. We treated a 14-year-old girl with relapsing polychondritis who had severe disease involving the pinnae, nasal cartilage, inner ear. sclera, kidneys and trachea which was not controlled by azathioprine 150 mg/day in combination with prednisoione 30 mg/day. Because of progressive tracheai stenosis, treatment was changed to cyclosporin 5 mg/kg/day with improvement in all indicators of disease activity for 3 months. However, while on this dose of cyclosporin and prodnisolone, the tracheai stenosis progressed, with collapse from 8 to 4 mm in diameter. Changing to cyclophosphamide 150 mg/day and prednisoione 50 mg/day arrested this progression, and has controlled the disease for 2 years in reduced dosage.Svenson"s patient, referred to by Anstey et al..^ was described in more detail elsewhere' and also had life-threatening pulmonary disease with tracheai stenosis, despite treatment with azathioprine and subsequently with cyclophosphamide 100 mg/day and prednisoione 60 mg/day. She rapidly improved with cyclosporin 15 mg/kg/day and showed a fall in anticoiiagen 11 antibodies with both cyclophosphamide and cyclosporin. She was eventually controlled on cyclosporin 6 mg/kg/ day and her disease remained inactive 2 years later. A further patient with tracheai stenosis was reported by Rogerson ct ai* Their patient was treated with high-dose intravenous prednisoione. and steroid reduction was made possible by the addition of eyclosporin (i-2 5 mg/kg/day. The authors cite a further case showing a dramatic response to cyclosporin. A recently described patient with cardiac involvement responded well to cyclosporin.^ However, one of the patients described by 1 Jm and Chan with tracheai stenosis was unresponsive to cyclosporin and other immunosuppressants. including azathioprine and cyclophosphamide.'' Finally, a patient who failed to respond to prednisoione (up to f)0 mg/ day) in combination with azathioprine 2 5 mg/kg/day. cyclophosphamide 2 S mg/kg/day and cyclosporin 4 mg/kg/day, responded to anti-CD4 monoclonal antibody 25 mg/day for 7 days." Two courses of treatment blocked disease progression for 12 months. It appears that cyclosporin has much to offer for relapsing poiychondritis and for some patients is the best choice. For others, cyciophosphamide has been more effective, and antiCD4 antibodies look promising. The condition has a significant mortality and for the patient with adverse prognostic indicators'* immunosuppressive drugs have an important place. There is insufficient evidence to show which of these agents is preferable.
300
Department of Dermatology. Aberdeen Rmial Infirmary. Voresterh'iU. Aberdeen AB9 27.R. U.K.
A.D.ORMEROD L.J.CLARX*
"Heatson Institute for Cancer Research. Wolfson Laboratory for Molecular Pathology. Bcarsden. Glasgow. U.K.
References 1 Anstey A. Mayou S. Morgan K el al Relapsing polychondritis: autoimmunity to type il collagen and treatment with cyclosporin A. BrIDermatol 1991; 125: 588-91. 2 Clarke L. Wakeel RA, Omierod AD. Relapsing polychondritis; two patients with tracheai stenosis and inner ear involvement. / Larifnfjo! Otologii 1992: (in press). 3 Svenson KL. Holmdahl R. Klareskog L et al. Cyclosporin A treatment in a case of relapsing polychondritis. Scand j Hheiimatol 1984; 1 J: 329-33. 4 Rogerson ME. Higgins VM. (Jodfrey RC. Tracheai stenosis due to relapsing polychondritis in rheumatoid arthritis. Thorax 198 7; 42: 905-6. 5 Mayer MC. Visconti M. Hassano P. Calloro V. l.a polycondrite ricorrente. Descrizione di un caso con peculiare interessaniento cardiaco. Retenti Profi Med 1991; 82: 83-5. 6 Lim MC. Chan HL. Relapsing polychondritis—a report on two Chinese patients with severe costal chondritis. Ann Acad Med Singapore 1990: 19: 396-403. 7 Van Der Lubbe PA, Miltenburg AM. Breedveld FC. Anti-CD4 monoclonal antibody for relapsing polychondritis. Lancet 1991; JJ7: 1 349. H Michet Cl \r. McKenna CH, Luthra HS, O'Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Int Med 1986; 104: 74-8.
Visual impairment secondary to rosacea SIR. Rosacea is a chronic muitiphasic vascular disorder of the skin. Its precise aetiology is unknown, hut environmental or endogenous agents may initiate damage to connective tissue around upper dermal blood vessels,' resulting in unsupported and dilated leaky vessels. The process is manifest clinically by erythema, telangiectasia, papules, pustules, chronic facia! oedema, and sebaceous gland hyperplasia.- Ocular involvement in rosacea is also felt to be vascular, or to have a vascular basis.' We describe a patient with unusual rosacea of the eyelids, which caused ptosis and signiticant impairment of vision. An 80-year-old man presented with a 3'year history of progressive swelling and aching of his eyelids. The swelling on the right totally occluded his eye and he had intermittent complete ptosis on the left. F.xamination revealed an elderly man with marked kyphosis. There was gross bulbous swelling of the right eyelid causing a complete right ptosis. He also had
Correspondence Relapsing polychondritis—treatment with cyclosporin A SIR. The case report by Anstey et ai' was interesting and raises the question of the place of cycfosporin in the treatment of relapsing polychondritis. Because of the rarity of this disease. new information on treatment is only likely to be acquired through such case reports. We would like to add some information based on personal experience in treating a patient, and from the sparse literature on this subject. We treated a 14-year-old girl with relapsing polychondritis who had severe disease involving the pinnae, nasal cartilage, inner ear. sclera, kidneys and trachea which was not controlled by azathioprine 150 mg/day in combination with prednisoione 30 mg/day. Because of progressive tracheai stenosis, treatment was changed to cyclosporin 5 mg/kg/day with improvement in all indicators of disease activity for 3 months. However, while on this dose of cyclosporin and prodnisolone, the tracheai stenosis progressed, with collapse from 8 to 4 mm in diameter. Changing to cyclophosphamide 150 mg/day and prednisoione 50 mg/day arrested this progression, and has controlled the disease for 2 years in reduced dosage.Svenson"s patient, referred to by Anstey et al..^ was described in more detail elsewhere' and also had life-threatening pulmonary disease with tracheai stenosis, despite treatment with azathioprine and subsequently with cyclophosphamide 100 mg/day and prednisoione 60 mg/day. She rapidly improved with cyclosporin 15 mg/kg/day and showed a fall in anticoiiagen 11 antibodies with both cyclophosphamide and cyclosporin. She was eventually controlled on cyclosporin 6 mg/kg/ day and her disease remained inactive 2 years later. A further patient with tracheai stenosis was reported by Rogerson ct ai* Their patient was treated with high-dose intravenous prednisoione. and steroid reduction was made possible by the addition of eyclosporin (i-2 5 mg/kg/day. The authors cite a further case showing a dramatic response to cyclosporin. A recently described patient with cardiac involvement responded well to cyclosporin.^ However, one of the patients described by 1 Jm and Chan with tracheai stenosis was unresponsive to cyclosporin and other immunosuppressants. including azathioprine and cyclophosphamide.'' Finally, a patient who failed to respond to prednisoione (up to f)0 mg/ day) in combination with azathioprine 2 5 mg/kg/day. cyclophosphamide 2 S mg/kg/day and cyclosporin 4 mg/kg/day, responded to anti-CD4 monoclonal antibody 25 mg/day for 7 days." Two courses of treatment blocked disease progression for 12 months. It appears that cyclosporin has much to offer for relapsing poiychondritis and for some patients is the best choice. For others, cyciophosphamide has been more effective, and antiCD4 antibodies look promising. The condition has a significant mortality and for the patient with adverse prognostic indicators'* immunosuppressive drugs have an important place. There is insufficient evidence to show which of these agents is preferable.
300
Department of Dermatology. Aberdeen Rmial Infirmary. Voresterh'iU. Aberdeen AB9 27.R. U.K.
A.D.ORMEROD L.J.CLARX*
"Heatson Institute for Cancer Research. Wolfson Laboratory for Molecular Pathology. Bcarsden. Glasgow. U.K.
References 1 Anstey A. Mayou S. Morgan K el al Relapsing polychondritis: autoimmunity to type il collagen and treatment with cyclosporin A. BrIDermatol 1991; 125: 588-91. 2 Clarke L. Wakeel RA, Omierod AD. Relapsing polychondritis; two patients with tracheai stenosis and inner ear involvement. / Larifnfjo! Otologii 1992: (in press). 3 Svenson KL. Holmdahl R. Klareskog L et al. Cyclosporin A treatment in a case of relapsing polychondritis. Scand j Hheiimatol 1984; 1 J: 329-33. 4 Rogerson ME. Higgins VM. (Jodfrey RC. Tracheai stenosis due to relapsing polychondritis in rheumatoid arthritis. Thorax 198 7; 42: 905-6. 5 Mayer MC. Visconti M. Hassano P. Calloro V. l.a polycondrite ricorrente. Descrizione di un caso con peculiare interessaniento cardiaco. Retenti Profi Med 1991; 82: 83-5. 6 Lim MC. Chan HL. Relapsing polychondritis—a report on two Chinese patients with severe costal chondritis. Ann Acad Med Singapore 1990: 19: 396-403. 7 Van Der Lubbe PA, Miltenburg AM. Breedveld FC. Anti-CD4 monoclonal antibody for relapsing polychondritis. Lancet 1991; JJ7: 1 349. H Michet Cl \r. McKenna CH, Luthra HS, O'Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Int Med 1986; 104: 74-8.
Visual impairment secondary to rosacea SIR. Rosacea is a chronic muitiphasic vascular disorder of the skin. Its precise aetiology is unknown, hut environmental or endogenous agents may initiate damage to connective tissue around upper dermal blood vessels,' resulting in unsupported and dilated leaky vessels. The process is manifest clinically by erythema, telangiectasia, papules, pustules, chronic facia! oedema, and sebaceous gland hyperplasia.- Ocular involvement in rosacea is also felt to be vascular, or to have a vascular basis.' We describe a patient with unusual rosacea of the eyelids, which caused ptosis and signiticant impairment of vision. An 80-year-old man presented with a 3'year history of progressive swelling and aching of his eyelids. The swelling on the right totally occluded his eye and he had intermittent complete ptosis on the left. F.xamination revealed an elderly man with marked kyphosis. There was gross bulbous swelling of the right eyelid causing a complete right ptosis. He also had
