Anti-SRP antibody related inflammatory myositis: A heterogeneous group of necrotizing myositis

Jeet Hemantkumar Patel, Lalit Duggal, Neeraj Jain, Gurbir Bhandari
DOI: 10.15305/ijrci/v6i1/261

Abstract

Anti-SRP antibody related necrotizing myositis is a rapidly progressive, severe, proximal muscle weakness with atrophy. The present study deals with two cases with poor prognostic course, concluding anti-SRP antibody related myositis as a heterogeneous group of inflammatory myositis. One patient had subacute onset proximal muscle weakness of both lower limbs, raised CPK, and biopsy showing typical changes of anti-SRP related necrotizing myositis. He responded well to steroids and methotrexate.  The other patient had 3 months history of subacute onset weakness of both lower limb and was admitted with quadriparesis, bulbar palsy, aspiration pneumonia and respiratory distress. Even after treatment with intravenous immunoglobulin, broad spectrum antibiotics, steroids, and ventilatory support, the patient succumbed to the illness


Citation

Patel JH, Duggal L, Jain N, Bhandari G. IJRCI. 2018;6(1):CS3 DOI: 10.15305/ijrci/v6i1/261

Keywords

anti-SRP myositis, necrotising myositis, immunosuprresants, heterogeneous

References

1. T Miller, M Al-Lozi, G Lopate, and A Pestronk - Myopathy with antibodies to the signal recognition particle: clinical and pathological features. J Neurol Neurosurg Psychiatry. 2002 Oct; 73(4): 420–428.

2. Hengstman GJ, ter Laak HJ, Vree Egberts WT. Anti-signal recognition particle autoantibodies: marker of a necrotizing myopathy. Ann Rheum Dis. 2006; 65: 1635-8.

3. Bryan R. Whelan and David A. Isenberg - Poor response of anti-SRP-positive idiopathic immune myositis to B-cell depletion. Rheumatology. 2009; 48 (5): 594-595.

4. Brouwer R, Hengstman GJ, Vree Egberts W, et al. Autoantibody profiles in the sera of European patients with myositis. Ann Rheum Dis 2001;60:116-23.

5. Kao AH, Lacomis D, Lucas M, et al. Anti signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy. Arthritis Rheum 2004;50:209-15.

6. Suzuki S, Nishikawa A, Kuwana M, et al. Inflammatory myopathy with anti-signal recognition particle antibodies: case series of 100 patients. Orphanet Journal of Rare Diseases. 2015;10:61.

7. Arlet JB, Dimitri D, Pagnoux C, et al. Marked efficacy of a therapeutic strategy associating prednisone and plasma exchange followed by rituximab in two patients with refractory myopathy associated with antibodies to the signal recognition particle (SRP). Neuromusc Disord 2006; 16: 334-6.

8. Noss EH, Hausner-Sypek DL, Weinblatt. Rituximab as therapy for refractory polymyositis and dermatomyositis. J Rheumatol. 2006; 33: 1021-6.

9. Oddis CV. Idopathic inflammatory myopathy: management and prognosis. Rheum Dis Clin North Am 1994;20:811-26.

10. Srivastava P, Dwivedi S, Misra R. Myositis specific and myositis associated antibodies in Indian patients with inflammatory myositis. Rheumatol Int. 2016;36:935-43.

11. Mathew A, Francis I, John A, et al. Autoantibody Profile and Associated Clinical Outcomes in an Indian Population with Myositis. Rheumatology. 2015;54:122.