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|Year : 2021 | Volume
| Issue : 5 | Page : 1391-1393
Polymyositis Presenting with Respiratory Symptoms
Yashpal Singh1, Sanghamitra Laskar1, Manish Mittal1, Nadia Shirazi2, Sumit Gupta3
1 Department of Neurology, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India
2 Department of Pathology, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India
3 Department of Medicine, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India
|Date of Submission||03-Jun-2017|
|Date of Acceptance||08-Aug-2019|
|Date of Web Publication||30-Oct-2021|
Department of Neurology, Himalayan Institute of Medical Sciences, Jolly Grant, Dehradun - 248 140, Uttarakhand
Source of Support: None, Conflict of Interest: None
Polymyositis is a group of muscle disease characterised by progressive muscle inflammation and predominantly muscle weakness. It usually presents subacutely with proximal weakness and mild diffuse muscular pain. Some patients have atypical presentation like early respiratory difficulty, Motor neuron disease (MND), or isolated dysphagia which leads to delay in diagnosis and treatment. We present one such case.
Keywords: Interstial lung Disease (ILD), myasthenia gravis, polymyositis, weakness
Key Message: In cases of atypical presentation of inflammatory myopathy, the clinical correlation of any positive investigation like repeated increased CPK level may help in early diagnosis and treatment
|How to cite this article:|
Singh Y, Laskar S, Mittal M, Shirazi N, Gupta S. Polymyositis Presenting with Respiratory Symptoms. Neurol India 2021;69:1391-3
| » Case Report|| |
A 36-year-old man, presented to us with one and half year history of progressive nonspecific generalised weakness and fatigue. His problem started with low grade pyrexia and persistent cough which lasted for more than three months. At that time, he was worked up for tubercular infection which was negative and possibility of interstial lung disease (ILD) was considered. After few weeks, he developed pain with morning stiffness of joints in both hands. He took oral steroids for few months that resulted in some improvement in his symptoms of pain and weakness. But there was no relief of cough.
Nine months back patient started taking Ayurvedic medications for joint pain and cough. Patient got some relief with that treatment also. Six months back while on ayurvedic treatment, his weakness worsened and he developed fatigue, difficulty in gripping and inability to lift objects. He also developed ptosis in both eyes. His weakness further worsened after an episode of diarrhoea. After few days he became almost bed ridden. Patient was admitted in another tertiary care government hospital under pulmonary medicine department where he was worked up for ILD which was negative. His pulmonary function test (PFT) showed severe restrictive disease. He was advised for neurology opinion, but patient left that hospital and his general condition continued to deteriorate. He moved to another hospital where he underwent five sessions of plasmapheresis, keeping a possibility of myasthenia gravis in crisis. Although his condition improved but his work up for myasthenia gravis including anti-choline-esterase (AcHR) antibody, anti-MuSK (muscle specific kinase) antibody test, and repetitive nerve stimulation were negative. His nerve conduction studies (NCS) done at that time was interpreted as motor axonopathy in proximal and distal distribution based on relatively generalized low compound muscle action potential (CMAP) amplitude. Then he was discharged with probable diagnosis of acute motor axonal neuropathy (AMAN) variety of Gullain Barre syndrome (GBS) after some improvement with plasmapheresis.
On this admission, he presented to us with severe generalized weakness and respiratory difficulty. Patient was quadreparetic and admitted in ICU. Within 2 hours he was intubated and put on ventilator support. Patient was conscious and was able to follow verbal commands. He had motor power of 2/5 in all limbs and reflexes were depressed. There was no muscle atrophy of any of the limbs. He had bilateral mild ptosis with no extraocular muscle weakness. There was no sensory deficeit. The myasthenia crisis was ruled out by a negative response to neostigmine and normal repetitive nerve stimulation test. The laboratory investigations showed elevated muscle enzymes: creatinine phosphokinase (CPK) – 996 IU/L and lactate dehydrogenase (LDH) of 300 U/L with raised C reactive protein (CRP) of 4.5 mg/L and High erythrocyte sedimentation rate (ESR) of 40. His previous investigations showed high CPK levels (1097 to 4750 IU) [Table 1].
Needle electromyography (EMG) study revealed complex, low voltage muscle unit action potentials (MUAP) of short duration. The recruitment pattern was early and full. Muscle biopsy was done which showed degenerating and regenerating muscle fibers with endomyosial and perivascular lymphocytic infiltrates suggestive of myositis [Figure 1]. Patient was put on high dose intravenous methyl prednisolone. His condition started improving. He was extubated after 4 days and his power improved to 3/5 in all limbs. He was taken out of ICU. He was discharged with much improved condition on oral steroids.
|Figure 1: Photomicrograph 1 (jpeg 3); Partly atrophic muscle fibres surrounded by loose fibrofatty tissue and mild lymphocytic infiltration (H and E. 200×)|
Click here to view
| » Discussion|| |
Polymyositis is an idiopathic subacute or chronic and symmetrical weakness of proximal limb and trunk muscle without dermatitis. Some patients of polymyositis present with predominantly respiratory muscle involvement leading to respiratory failure.
Polymyositis may be associated with various systemic diseases like systemic lupus erythematosus (SLE) and mixed connective tissue disease. Some cases are also associated with malignancy.
Annual incidence rates of idiopathic inflammatory myopathies increases with age, ranging from 2.5 × 10-6 in people under 15 years of age to 10.5 × 10-6 in people over 65 years of age. These patients may present with initial symptoms unrelated to muscle disease which may lead to delay in diagnosis and treatment. Polymyositis can present initially with nonspecific generalized weakness and fatigue. Delay in diagnosis of polymyositis can be due to atypical presentation like early respiratory difficulty mimicking ILD or MND like presentation or isolated dysphagia.,, Myasthenia Gravis can also exist with Polymyositis and our patient had some features of myasthenia like ptosis but electrophysiological and antibody tests were negative Some cases also present with normal CPK level in early disease or CPK levels may normalise in some patients due to steroid therapy which may lead to delayed diagnosis, Respiratory involvement in polymyositis can be due associated connective tissue disorder, respiratory muscle involvement due to myopathy or infection.,, Most of cases early respiratory symptoms are accompanied with or soon followed by typical muscle weakness Treatment of polymyositis consists of high dose of steroids. Plasmapheresis is not used in treatment of polymyositis. Some open label studies have shown some muscle strength improvement in most of patients.
Our patient initially presented with respiratory complains along with fatigue and generalised weakness, but it was almost 8 months before he had features of myopathy that also along with symmetrical ptosis and it was nearly 18 months after onset of initial symptoms that he was diagnosed with polymyositis. So, in our case there was a delay in diagnosis may be due to initial respiratory symptoms, fatigue, generalized weakness, and myasthenia-like presentation. Third, though he had raised CK levels, he was being worked for myasthenia and AMAN variant GBS because of his atypical presentation and as he showed some improvement with plasmapheresis.
This case clearly shows in case of atypical presentation of inflammatory myopathy, the importance of clinical correlation of any positive investigation as early treatment in cases of polymyositis results in higher remission, shorter treatment period and low complication rates.
| » Conclusion|| |
Inflammatory myopathy like polymyositis can present with initial respiratory symptoms and delay in diagnosis and adequate treatment can be prevented by keeping broad version approach.
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Conflicts of interest
There are no conflicts of interest.
| » References|| |
Ropper AH, Samuels MA, Klein JP. Adams and Victors. McGraw Hill, Education 10th
ed. 2014; p. 1418.
Maclean J, Singh RB, Sayeed ZA. Polymyositis presenting with respiratory failure. Indian J Chest Dis Allied Sci 2011;53:229-31.
Dalakas MC. Retrovirus and inflammatory myopathies in humans and primates. Baillieres Clin Neurol 1993;2:659-91.
Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al
. Frequency of specific cancer types in dermatomyositis and polymyositis: A population-based study. Lancet 2001;357:96-100.
Mastaglia FL, Phillips BA. Idiopathic inflammatory myopathies: Epidemiology, classification, and diagnostic criteria. Rheum Dis Clin North Am 2002;28:723-41.
Anić B, Cerovec M. Polymyositis/dermatomyositis--clinical picture and treatment. Reumatizam 2012;59:44-50.
Ryan A, Nor AM, Costigan D, Foley-Nolan D, El-Rafie A, Farrell MA, et al
. Polymyositis masquerading as motor neuron disease. Arch Neurol 2003;60:1001-3.
Palace J, Losseff N, Clough C. Isolated dysphagia due to polymyositis. Muscle Nerve 1993;16:680-1.
Paik JJ, Corse AM, Mammen AL. The co-existence of myasthenia gravis in patients with myositis: A case series. Semin Arthritis Rheum 2014;43:792-6.
Gran JT, Myklebust G, Johansen S. Adult idiopathic polymyositis without elevation of creatine kinase. Case report and review of the literature. Scand J Rheumatol 1993;22:94-6. Review.
Volochayev R, Csako G, Wesley R, Rider LG, Miller FW. Laboratory test abnormalities are common in polymyositis and dermatomyositis and differ among clinical and demographic Groups. Open Rheumatol J 2012;6:54-63.
Clawson K, Oddis CV. Adult respiratory distress syndrome in polymyositis patients with the anti-Jo-1 antibody. Arthritis Rheum 1995;38:1519-23.
Blumbergs PC, Byrne E, Kakulas BA. Polymyositis presenting with respiratory failure. J Neurol Sci 1984;65:221-9.
Chen IJ, Tsai WP, Wu YJJ, Luo SF, Ho HH, Liou LB, et al
. Infections in polymyositis and dermatomyositis: Analysis of 192 cases. Rheumatology (Oxford) 2010;49:2429-37.
Choy EHS, Isenberg DA. Polymyositis Presenting with Respiratory Symptoms. Rheumatology (Oxford) 2002;41:7-13.
Naji P, Shahram F, Nadji A, Davatchi F. Effect of early treatment in polymyositis and dermatomyositis. Neurol India 2010;58:58-61.
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