RETINOPATHY IN ADULT AMYOPATHIC DERMATOMYOSITIS Kazuki Hotta, MD
A 53-year-old Japanese woman with retinopathy in adult amyopathic dermatomyositis is presented. The case is the eldest reported to date and is an extremely rare case corresponding to the second peak in the dermatomyositis age distribution.
From the Department of Ophthalmology, Kameda Medical Center, Chiba, Japan.
unremarkable, but fundus examination of both eyes showed multiple cotton-wool spots and retinal vein dilatation with vitreous haze indicating severe retinal vessel inflammation (Fig. 1A). Fluorescein angiography indicated faint dye leakage from retinal veins in both eyes (Fig. 2A). Three months after the first ophthalmologic examination, vision was restored to 20/20 in both eyes, and multiple cotton-wool spots and retinal vein dilatation were resolved gradually and spontaneously (Fig. 1B). Fluorescein angiography also showed an almost normal fundus appearance without any dye leakage or filling defect, except for multiple small fluorescence blockages caused by the new appearance of small pigment clumps (Fig. 2B). Through the 12-month follow-up, myopathic signs were absent.
D
ermatomyositis is a collagen vascular disease with an autoimmune background that involves striated muscles with characteristic cutaneous lesions. Posterior ocular manifestations, such as cotton-wool spots, occur occasionally in children or young adults with dermatomyositis, whereas they are extremely rare in elderly patients.1 We describe a middle-aged patient who had retinopathy with amyopathic dermatomyositis, a variant of dermatomyositis.2
Discussion To our knowledge, this is the first description of a patient developing retinopathy complicated by amyopathic dermatomyositis. Amyopathic dermatomyositis is a variant of dermatomyositis characterized by a typical skin rash but without muscle abnormalities. This concept is supported by both family history and genetic studies.2 The age distribution of dermatomyositis shows a bimodal curve with peaks at 5–15 and 50 – 60 years of age.3 The amyopathic form is more common in adults than in children.2 Interestingly, all cases of retinopathy with dermatomyositis reported previously in the English-language literature occurred in children or young adults. Yeo et al4 and Chu-Lee et al5 reported adult cases; their patients were a 19-year-old woman and 23-year-old man, respectively. Only Enoki et al,6 in the Japanese literature, reported a middle-aged case of a 52-year-old woman with dermatomyositis who had severe visual disturbance and died of respiratory failure due to interstitial pneumonia. Therefore, to our knowledge, our patient is the eldest described to date, and this case is extremely rare corresponding to the second peak in the dermatomyositis age distribution. Although it is known that interstitial pneumonia has an acute course and poor prognosis in amyopathic dermatomyositis,2 the patient described here re-
Case Report A 53-year-old Japanese woman was admitted to our hospital because of deterioration of interstitial pneumonia on October 27, 2002. The patient presented with red and scaly skin plaques over both elbows and a heliotrope rash around the eyes; lung fibrosis was noted by plain chest roentgenography and chest computed tomography. The patient had no signs of proximal muscle weakness or arthralgia of the wrists or knees. Electromyography and muscle biopsy showed no typical myopathic signs. The creatine phosphokinase level was also normal at 47 IU/L. However, analysis of biopsy tissue from the skin lesion revealed superficial and deep perivascular dermatitis of the vacuolar type, compatible with dermatomyositis. Jo-1 testing was positive. However, results of other serological tests were normal. Therefore, a diagnosis of interstitial pneumonia with amyopathic dermatomyositis was made. After induction of chemotherapy with pulsed intravenous methylprednisolone (1,000 mg for 3 days) and subsequent oral prednisolone (50 mg/d) and cyclosporin A (150 mg/d), the respiratory symptoms and interstitial shadows were gradually reduced. The general condition of the patient was stabilized, and treatment with a reduced dosage of oral prednisolone (25 mg/d) and cyclosporin A (75 mg/d) was maintained; however, the patient reported mild blurred vision in both eyes for 1 week at referral to our eye clinic on June 17, 2003. Ophthalmologic testing disclosed best-corrected visual acuity of 20/25 in both eyes, and intraocular pressure was 18 mmHg in both eyes. The anterior segments were Reprint requests: Kasuki Hotta, Department of Ophthalmology, Kameda Medical Center, 929 Higashi, Kamogawa, Chiba 2968602, Japan; e-mail: [email protected]
77
78
RETINAL CASES & BRIEF REPORTSℜ
●
2007
●
VOLUME 1
●
NUMBER 2
sponded well to chemotherapy with prednisolone and cyclosporin A. Retinopathy in this case appeared after remission of interstitial pneumonia with chemotherapy and resolved spontaneously within a few months. Thus, further observation of the fundus is recommended for patients with dermatomyositis even after remission of the general condition. Key words: amyopathic dermatomyositis, retinopathy, cotton-wool spots, interstitial pneumonia. References 1.
2. Fig. 1. At initial presentation, fundus examination showed multiple cotton-wool spots and retinal vein dilatation with vitreous haze (A). Three months after initial presentation, multiple cotton-wool spots and retinal vein dilatation were resolved (B). Fig. 2. Fluorescein angiography indicated faint dye leakage from retinal veins (A). Three months after initial presentation, fluorescein angiography also showed an almost normal fundus appearance except for multiple small fluorescence blockages caused by small pigment clumps (B).
3. 4.
5. 6.
Harrison SM, Frenkel M, Grossman BJ, Matalon R. Retinopathy in childhood dermatomyositis. Am J Ophthalmol 1973; 76:786–790. Olsen NJ, Park JH, King Jr LE. Amyopathic dermatomyositis. Curr Rheumatol Rep 2001;3:346–351. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;292:344–347. Yeo LMW, Swaby DSA, Situnayake RD, Murray PI. Irreversible visual loss in dermatomyositis. Br J Rheumatol 1995;34: 1179–1181. Chu-Lee A, Stoller G, Jaffe IA. Retinopathy in adult dermatomyositis. J Rheumatol 1995;22:2372–2373. Enoki T, Nakagami T, Murakami A, et al. A case of dermatomyositis with severe retinopathy in a patient who died of acute interstitial pneumonia. Nippon Ganka Gakkai Zasshi 1997; 101:441–445.
A 53-year-old Japanese woman with retinopathy in adult amyopathic dermatomyositis is presented. The case is the eldest reported to date and is an extremely rare case corresponding to the second peak in the dermatomyositis age distribution.
From the Department of Ophthalmology, Kameda Medical Center, Chiba, Japan.
unremarkable, but fundus examination of both eyes showed multiple cotton-wool spots and retinal vein dilatation with vitreous haze indicating severe retinal vessel inflammation (Fig. 1A). Fluorescein angiography indicated faint dye leakage from retinal veins in both eyes (Fig. 2A). Three months after the first ophthalmologic examination, vision was restored to 20/20 in both eyes, and multiple cotton-wool spots and retinal vein dilatation were resolved gradually and spontaneously (Fig. 1B). Fluorescein angiography also showed an almost normal fundus appearance without any dye leakage or filling defect, except for multiple small fluorescence blockages caused by the new appearance of small pigment clumps (Fig. 2B). Through the 12-month follow-up, myopathic signs were absent.
D
ermatomyositis is a collagen vascular disease with an autoimmune background that involves striated muscles with characteristic cutaneous lesions. Posterior ocular manifestations, such as cotton-wool spots, occur occasionally in children or young adults with dermatomyositis, whereas they are extremely rare in elderly patients.1 We describe a middle-aged patient who had retinopathy with amyopathic dermatomyositis, a variant of dermatomyositis.2
Discussion To our knowledge, this is the first description of a patient developing retinopathy complicated by amyopathic dermatomyositis. Amyopathic dermatomyositis is a variant of dermatomyositis characterized by a typical skin rash but without muscle abnormalities. This concept is supported by both family history and genetic studies.2 The age distribution of dermatomyositis shows a bimodal curve with peaks at 5–15 and 50 – 60 years of age.3 The amyopathic form is more common in adults than in children.2 Interestingly, all cases of retinopathy with dermatomyositis reported previously in the English-language literature occurred in children or young adults. Yeo et al4 and Chu-Lee et al5 reported adult cases; their patients were a 19-year-old woman and 23-year-old man, respectively. Only Enoki et al,6 in the Japanese literature, reported a middle-aged case of a 52-year-old woman with dermatomyositis who had severe visual disturbance and died of respiratory failure due to interstitial pneumonia. Therefore, to our knowledge, our patient is the eldest described to date, and this case is extremely rare corresponding to the second peak in the dermatomyositis age distribution. Although it is known that interstitial pneumonia has an acute course and poor prognosis in amyopathic dermatomyositis,2 the patient described here re-
Case Report A 53-year-old Japanese woman was admitted to our hospital because of deterioration of interstitial pneumonia on October 27, 2002. The patient presented with red and scaly skin plaques over both elbows and a heliotrope rash around the eyes; lung fibrosis was noted by plain chest roentgenography and chest computed tomography. The patient had no signs of proximal muscle weakness or arthralgia of the wrists or knees. Electromyography and muscle biopsy showed no typical myopathic signs. The creatine phosphokinase level was also normal at 47 IU/L. However, analysis of biopsy tissue from the skin lesion revealed superficial and deep perivascular dermatitis of the vacuolar type, compatible with dermatomyositis. Jo-1 testing was positive. However, results of other serological tests were normal. Therefore, a diagnosis of interstitial pneumonia with amyopathic dermatomyositis was made. After induction of chemotherapy with pulsed intravenous methylprednisolone (1,000 mg for 3 days) and subsequent oral prednisolone (50 mg/d) and cyclosporin A (150 mg/d), the respiratory symptoms and interstitial shadows were gradually reduced. The general condition of the patient was stabilized, and treatment with a reduced dosage of oral prednisolone (25 mg/d) and cyclosporin A (75 mg/d) was maintained; however, the patient reported mild blurred vision in both eyes for 1 week at referral to our eye clinic on June 17, 2003. Ophthalmologic testing disclosed best-corrected visual acuity of 20/25 in both eyes, and intraocular pressure was 18 mmHg in both eyes. The anterior segments were Reprint requests: Kasuki Hotta, Department of Ophthalmology, Kameda Medical Center, 929 Higashi, Kamogawa, Chiba 2968602, Japan; e-mail: [email protected]
77
78
RETINAL CASES & BRIEF REPORTSℜ
●
2007
●
VOLUME 1
●
NUMBER 2
sponded well to chemotherapy with prednisolone and cyclosporin A. Retinopathy in this case appeared after remission of interstitial pneumonia with chemotherapy and resolved spontaneously within a few months. Thus, further observation of the fundus is recommended for patients with dermatomyositis even after remission of the general condition. Key words: amyopathic dermatomyositis, retinopathy, cotton-wool spots, interstitial pneumonia. References 1.
2. Fig. 1. At initial presentation, fundus examination showed multiple cotton-wool spots and retinal vein dilatation with vitreous haze (A). Three months after initial presentation, multiple cotton-wool spots and retinal vein dilatation were resolved (B). Fig. 2. Fluorescein angiography indicated faint dye leakage from retinal veins (A). Three months after initial presentation, fluorescein angiography also showed an almost normal fundus appearance except for multiple small fluorescence blockages caused by small pigment clumps (B).
3. 4.
5. 6.
Harrison SM, Frenkel M, Grossman BJ, Matalon R. Retinopathy in childhood dermatomyositis. Am J Ophthalmol 1973; 76:786–790. Olsen NJ, Park JH, King Jr LE. Amyopathic dermatomyositis. Curr Rheumatol Rep 2001;3:346–351. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;292:344–347. Yeo LMW, Swaby DSA, Situnayake RD, Murray PI. Irreversible visual loss in dermatomyositis. Br J Rheumatol 1995;34: 1179–1181. Chu-Lee A, Stoller G, Jaffe IA. Retinopathy in adult dermatomyositis. J Rheumatol 1995;22:2372–2373. Enoki T, Nakagami T, Murakami A, et al. A case of dermatomyositis with severe retinopathy in a patient who died of acute interstitial pneumonia. Nippon Ganka Gakkai Zasshi 1997; 101:441–445.
