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| LETTER TO EDITOR |
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| Year : 2013 | Volume
: 61
| Issue : 1 | Page : 83-84 |
Antisynthetase syndrome with stroke
KP Divya, Sajith Sukumaran, Sapna Erat Sreedharan, PN Sylaja
Comprehensive Stroke Care Program, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum 695 011, Kerala, India
| Date of Submission | 08-Feb-2012 |
| Date of Decision | 18-Dec-2012 |
| Date of Acceptance | 09-Dec-2012 |
| Date of Web Publication | 4-Mar-2013 |
Correspondence Address:
P N Sylaja
Comprehensive Stroke Care Program, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum 695 011, Kerala
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.108022
How to cite this article:
Divya K P, Sukumaran S, Sreedharan SE, Sylaja P N. Antisynthetase syndrome with stroke. Neurol India 2013;61:83-4 |
Sir,
Antisynthetase syndrome (ASS) is a rare autoimmune disease of unknown etiology. The hallmark of the disorder is the presence of serum autoantibodies, which recognize the aminoacyl-tRNA synthetases, a family of intracytoplasmic enzymes that play a role in protein synthesis. [1] The clinical characteristics of ASS include myositis, interstitial lung disease, and chronic articular involvement either alone or in combination. Involvement of various systems has been reported in ASS, neurological manifestations have not been reported previously. [2]
A 52-year-old female presented with acute onset right upper and lower limb weakness 8 hours after onset. She had history of polyarthritis and exertional dyspnea for which she was evaluated and diagnosed as interstitial lung disease and was on irregular treatment with steroids and immunosuppressants. She did not have history suggestive of photosensitivity, Raynaud's phenomenon or myalgia. General examination revealed "mechanic's hands" and respiratory system examination showed fine inspiratory crepitations. Neurological examination revealed conscious and mute patient with grade 3/5 power in right upper limb and grade 0/5 power in right lower limb with National Institute of Health Stroke Scale score of 17.
Computed tomography (CT) head showed acute infarct in left frontal region in the anterior cerebral artery (ACA) territory and CT-angiogram showed left ACA A3 territory distal occlusion [Figure 1]a and b. Serum antinuclear antibody (ANA) was positive and anti-Ro and anti-Jo-1 titres were high. Digital subtraction angiography (DSA) showed fusiform dilatation of A3 segments of bilateral ACA [Figure 1]c. CT chest showed features of interstitial lung disease [Figure 1]d. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were high. On the basis of the clinical features and investigative findings, the diagnostic possibility of ASS was considered. In view of the DSA findings, etiology of stroke was considered to be due to central nervous system (CNS) vasculitis secondary to ASS. As the disease was active as suggested by the raised ESR and CRP she was administered on IV methylprednisolone for 5 days followed by oral steroids and azathioprine. | Figure 1: (a) CT head showing a left frontal infarct, (b) CT angiogram showed occlusion of the left ACA A3 occlusion, (c) Digital substraction angiogram showed fusiform dilatation of A3 segments of bilateral ACA, (d) CT chest showed features of interstitial lung disease
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ASS affects mainly adult individuals with a female to male ratio of 2.3:1. Clinical manifestation of ASS is relatively homogeneous with one or more of the following features: Myositis, interstitial pulmonary disease, and articular involvement. The presence of fever, Raynaud's phenomenon, and "mechanical hands" may also be observed. [2] ASS can present antibodies against different aminoacyl tRNA synthetases. The most frequent is anti-Jo-1 against histidyl-tRNA synthetase. The presence of anti-Ro antibody has been associated to pulmonary fibrosis in ASS. [3] Practically all of the patients have muscle affection. But rare reports of absence of muscle involvement has been reported. [4],[5] Our patient had definite ASS. He had mechanic hand, interstitial lung disease, and serological markers of ASS and the disease was active as evidenced by raised ESR and CRP. However, he did not have features of myositis. A thorough literature search did not reveal any case reports of neurological involvement as in our patient. We report this case in view of the first documentation of CNS involvement in ASS as suggested by the evidence of vasculitis in the angiography. The treatment is mainly with corticosteroids and other immunosuppressive agents like cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, and tacrolimus.
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| 1. | Imbert-Masseau A, Hamidou M, Agard C, Grolleau JY, Chérin P. Antisynthetase syndrome. Joint Bone Spine 2003;70:161-8. 
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| 2. | Shinjo SK, Levy-Neto M. Anti-Jo-1 antisynthetase syndrome. Rev Bras Reumatol 2010;50:492-500.
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| 3. | Nishikai M, Reichlin M. Heterogeneity of precipitating antibodies in polymyositis and dermatomyositis. Characterization of the Jo-1 antibody system. Arthritis Rheum 1980;23:881-8.
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| 4. | Júdez Navarro E, Martinez Carretero M, Martínez-Jiménez GF. Antisynthetase syndrome without muscle involvement. Reumatol Clin 2007;3:276-7.
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| 5. | Climent-Albaladejo A, Saiz-Cuenca E, Rosique Roman J, Caballero-Rodriguez J, Galvez-Munoz J. Dermatomyositis sine myositis and antisynthetase syndrome. Joint Bone Spine 2002;69:72-5.
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[Figure 1]
| This article has been cited by | | 1 |
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| Monique Kowitz, Katrina Chakradeo, Ashleigh Hennessey, Penny Wolski | | BMJ Case Reports. 2021; 14(5): e240929 | | [Pubmed] | [DOI] | | | 2 |
Branch Retinal Vein Occlusion as a Rare Presentation of Antisynthetase Syndrome |
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| Swati Parida, Konstantinos T Tsaousis, Sundeep Deol, Vasilios Diakonis, Vasileios Konidaris | | Cureus. 2021; | | [Pubmed] | [DOI] | |
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