Volume 90, Issue 1 , Pages 170-172, January 2009
Functional Outcome in a Patient With an Acute Quadriparesis Secondary to Systemic Sclerosis: A Case Report
Article Outline
Abstract
Chernev I, Gustafson K, Medina-Bravo A. Functional outcome in a patient with an acute quadriparesis secondary to systemic sclerosis: a case report.
Scleroderma or systemic sclerosis (SSc) is a relatively uncommon disease. Although well-known for many years, research on appropriate physical therapy during all stages of myositis due to scleroderma is limited. We report the functional outcome in a patient with an acute quadriparesis secondary to diffuse SSc associated with extensive myositis. This 35-year-old black woman progressed to almost complete functional recovery in the course of 16 days of acute rehabilitation with combined physical therapy including resistive exercises. This case strongly suggests that a patient with diffuse SSc and associated myositis can undergo aggressive physical therapy in a monitored environment with good functional improvement and no worsening of the myositis.
Key Words: Exercises, Idiopathic inflammatory myopathies, Myositis, Quadriparesis, Rehabilitation, Scleroderma, systemic
List of Abbreviations: ADLs, activities of daily living, CK, creatine kinase, HTN, hypertension, MRI, magnetic resonance imaging, SSc, systemic sclerosis
SCLERODERMA OR SYSTEMIC SCLEROSIS is an autoimmune connective tissue disease characterized by involvement of multiple organ systems. Scleroderma is a relatively uncommon problem affecting only 200 to 300 per 1 million people in the United States, with approximately 12 to 20 newly diagnosed cases per million annually.1 The exact etiology of SSc is unclear. The skin and other systems, such as gastrointestinal, respiratory, renal, cardiovascular, and genitourinary are frequently involved. The symptoms result from inflammation and progressive tissue fibrosis and occlusion of the microvasculature, caused by excessive production and deposition of collagen. Severe alterations in small blood vessels of skin and internal organs, including fibrosis and perivascular cellular infiltration with activated T cells, are almost always present in scleroderma.2
The 2 main types of scleroderma are limited cutaneous SSc and diffuse cutaneous SSc.3 Limited cutaneous SSc involves areas distal to the elbow and knee but may also involve the face and neck. CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia [not all are needed to be called CREST]) syndrome is an older term used to describe this subset of limited cutaneous SSc. Diffuse cutaneous SSc refers to skin thickening on the trunk and proximal aspects of the extremities in addition to the face, with multiple organ systems involved. Factors that imply a more severe prognosis are: young age, African descent, rapid progression of skin symptoms, extent of skin involvement, anemia, increased erythrocyte sedimentation rate, pulmonary involvement, and renal involvement. There are many neuromuscular manifestations of SSc affecting the central and peripheral nervous systems as well as the muscular system.4
In this clinical case, we discuss a rare presentation of SSc with severe myopathy.
Case Description
Chief Complaint
A 35-year-old black woman with a known history of scleroderma was admitted to the hospital with acute onset of generalized myalgias and profound weakness in all 4 extremities to the extent of functional quadriparesis.
History
This patient was diagnosed 5 years earlier with diffuse cutaneous SSc, which progressed rapidly with extensive skin disease, interstitial lung disease with pulmonary HTN, gastroesophageal reflux disease, Raynaud's phenomenon, and HTN. Her disease was treated by immunosuppression with mycophenolate mofetil (CellCept). She was transported to the hospital by emergency medical service for 1-day acute onset of generalized myalgias and profound weakness, not feeling well, not eating well, and with nonexertional chest pain. Before admission, she had neither subjective weakness nor difficulty climbing stairs or getting up from a seated position. On admission in the emergency department, the patient's skin was cold and clammy, and she appeared diaphoretic but mentating appropriately. She reported some pain with swallowing but no sensation of food getting stuck in her throat. She did not complain of dyspnea and did not require mechanical ventilation.
Review of systems revealed no fever; no headache; no vision problems; no lightheadedness; no dyspnea or cough; no diarrhea, vomiting, constipation or abdominal pain; no dysuria or urinary frequency; and no paresthesias or hypoesthesias.
Medication list included mycophenolate mofetil (CellCept), pantoprazole (Flexeril), cyclobenzaprine HCl (Protonix), nifedipine, metoprolol, oxycodone, and hydroxizine. Previous medical history included pneumonia and sickle cell trait. There was no history of alcohol, cigarette, or drug use.
Review of the previous records revealed that the patient has positive antinuclear antibodies and negative anti-Smith, antiribonucleoprotein, and antitopoisomerase (antiscleroderma-70) antibodies.
On physical examination, the patient was alert and oriented. Vital signs indicated the patient was afebrile, tachycardic, and hypotensive. Skin was thickened with multiple hypo- and hyperpigmented spots across the body.
Neck was supple. Cardiac examination found no murmurs, no jugular venous distention, tachycardia, or bilateral asymmetric upper-extremity edema. Vascular pulses were palpable 1+ radial, dorsalis pedis, and posterior tibialis. Pulmonary examination showed no respiratory distress, and no accessory muscle use. Bibasilar rales were appreciated on auscultation. Abdomen was soft, nondistended, and nontender. Extremities were edematous, and bilateral sclerodactily. Muscle strength was 1–2/5 in the upper and lower extremities on manual muscle testing. Reflexes were absent in bilateral upper and lower extremities. The patient was unable to move her extremities against gravity. She was able to shrug her shoulders and move her head against gravity and with minimal resistance. Sensation to light touch and pinprick was maintained.
Labs showed a white blood cell count of 27 with 83% polymorphonuclears, hematocrit 50, platelets 420, creatinine 0.5, troponin 2.2, lactate 4.9, urinalysis negative, arterial blood gases 7.26/39/174/18 on supplemental oxygen, and CK 3319 with remarkable peak up to 14 to 124 (normal range, 23–240U/L) in the subsequent days. Chest radiograph showed bilateral increased reticular markings unchanged from previous examination, consistent with pulmonary fibrosis. Persistent blunting of the right costophrenic angle consistent with a small pleural effusion or pleural scaring.
Electrocardiogram revealed no ischemic changes.
The patient was admitted for presumed sepsis of unknown source due to immunosuppression with mycophenolate mofetil. She was started on antibiotics for broad-spectrum coverage. She was also started on high-dose steroids for myositis, and mycophenolate mofetil (Cellcept) was discontinued. Initially, she was given loading doses of methylprednisolone: 1000mg intravenously daily for 4 days, followed by 60mg intravenously daily for 3 days. On the seventh hospital day, intravenous methlyprednisolone was replaced with oral methylprednisolone 60mg daily, which was slowly tapered during the hospital course. Antibiotics were discontinued on hospital day 7 due to the negative infectious workup.
Transthoracic echocardiogram showed mild pulmonary HTN with pulmonary artery systolic pressure at 42mmHg and left ventricular ejection fraction 65%.
MRI of the thighs showed an abnormal T2 hyperintense signal within the musculature of the thighs bilaterally, predominantly within the anterior and posterior compartments of the thighs, consistent with extensive myositis. Adductor longus, gracilis and sartorius muscles were relatively spared. T2 hyperintense signal was also seen within subcutaneous tissue of the thighs bilaterally (fig 1).
Fig 1.
MRI of bilateral thighs showing extensive myositis with relative sparing of the medial muscle compartment.
On hospital day 11, the patient was transferred to acute rehabilitation for further treatment. The muscle strength examination on admission to the rehabilitation unit was still 1–2/5 in the upper and lower extremities. The CK at that time was 178U/L. A combined physical therapy program was started with a very fast improvement of muscle strength and function. This program consisted of 3 or more hours of therapy a day with 6 sessions a week. Physical and occupational therapy were equally involved in the program. Stretching exercises to prevent contractures as well as resistive training were the mainstay of the program. On discharge the patient had muscle strength 4–5/5 in all muscle groups. She was able to ambulate and to perform all basic ADLs. The CK showed a decreasing trend with 80U/L and 75U/L on subsequent laboratory results during her rehabilitation stay.
Follow-up MRI demonstrated significant interval improvement of myositis with residual mild T2 hyperintense signal in the anterior compartments of both thighs and moderate signal abnormality in the posterior compartments (fig 2).
Fig 2.
MRI of bilateral thighs showing resolving myositis.
Discussion
SSc is one of the most common connective tissue diseases associated with idiopathic inflammatory myopathies. This complication is also called overlap myositis. Idiopathic inflammatory myopathies are more often described in dermatomyositis, polymyositis, and inclusion body myositis. The course of the myositis can be chronic, polycyclic, or monophasic. Large studies of the role and type of exercises during the rehabilitative period of inflammatory myositis have not been done. Most of the published literature consists of case reports and small case series. Of these reports, most described dermatomyositis and polymyositis rehabilitation treatment and few were randomized double-blind controlled trials.5, 6, 7, 8, 9, 10
Although the clinical picture is similar in all types of myositis, the potential for recovery and the effect of different types of exercises in different types of myositis is still not clear. In the past, patients with idiopathic inflammatory myositis were discouraged from active exercises because of the fear of further muscular damage. In recent years, benefits of active exercises in all stages of inflammatory myositis without increased muscle inflammation and damage have been reported.6, 9 In our review of the literature, we did not find any other reported similar cases of an acute quadriparesis secondary to overlap myositis in a patient with diffuse SSc. In the course of 16 days of acute rehabilitation treatment, this patient regained most of her muscle strength and was able to ambulate and perform her basic ADLs. Of particular interest were the daily resistive exercises, which included supine exercises with weights, stationary bicycle, step-ups, upper-body ergometry, and upper-body progressive weightlifting exercises. The follow-up CK during the recovery period did not show any further damage to the muscles with the gradually increased intensity of physical therapy. Follow-up MRI on discharge showed resolving myositis (see fig 2). MRI was used as an aid in the diagnosis of myositis, and as per other reports it has become an important diagnostic modality in the diagnosis and response to treatment. MRI can also be used to evaluate muscle atrophy and fatty replacement, and thus serves as a measure of chronic muscle damage.10, 11 People with myositis may have extensive functional limitations and disability despite the appropriate pharmacologic treatment to reduce inflammatory damage to their muscles.12 Physical therapy with vigorous exercises to increase the muscle power and aerobic capacity with appropriate medical monitoring can substantially improve strength and functional outcome.
Lastly, patients with myositis of different etiology may respond differently to the more aggressive physical therapy.
Conclusions
This is a rare case of an acute quadriparesis due to extensive myositis secondary to advanced diffuse SSc in a patient with prior independent functional status. The patient progressed extremely well with combined physical therapy, including resistive exercises, range of motion exercises, as well as gross- and fine-motor training. In this case, steroid treatment and combined physical therapy in an acute rehabilitation setting substantially improved the outcome of this patient. This case suggests that a patient with diffuse SSc and associated myositis can undergo aggressive physical therapy in a monitored environment with good functional improvement and no worsening of the myositis. Further studies are needed to analyze the proper type of exercises, intensity, frequency, and timing of therapy in patients with myositis secondary to SSc. Correlation with respect to the severity of the disease is important to compare the effectiveness of the therapy.
References
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- Scleroderma. http://www.emedicine.com/med/TOPIC2076.HTMAccessed October 19, 2008
- . Physical rehabilitation, evidence-based examination, evaluation and intervention. In: Philadelphia: Elsevier; 2007;p. 87–116
- . Neuromuscular manifestations of systemic sclerosis (scleroderma). http://www.uptodate.com/online/content/topic.do?topicKey=sclerode/5826&selectedTitle=7∼150&source=search_resultAccessed February 14, 2008
- . Safety and efficacy of submaximal eccentric strength training for a subject with polymyositis. Arthritis Rheum. 2005;53:471–474
- . Resistive exercise in the rehabilitation of polymyositis/dermatomyositis. J Rheumatol. 1993;20:1340–1344
- . The role of exercise in the rehabilitation of idiopathic inflammatory myopathies. Curr Opin Rheumatol. 2005;17:164–171
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- . Isometric exercise increases strength and does not produce sustained creatinine phosphokinase increases in a patient with polymyositis. J Rheumatol. 1993;20:1399–1401
- . Quantitative assessments of the effects of a single exercise session on muscles in juvenile dermatomyositis. Arthritis Rheum. 2005;53:558–564
- . Why isn't my myositis patient getting better?. The Rheumatologist. 2008;2:1,18-19,22 http://media.wiley.com/assets/1443/10/THR0108Web.pdfAccessed October 20, 2008
- . Physical therapy to manage inflammatory muscle disease. The Rheumatologist. 2008;2:1,16-7 http://media.wiley.com/assets/1443/10/THR0108Web.pdfAccessed October 20, 2008
No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit on the authors or on any organization with which the authors are associated.
Reprints are not available from the authors.
PII: S0003-9993(08)01547-5
doi:10.1016/j.apmr.2008.06.033
© 2009 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.
Volume 90, Issue 1 , Pages 170-172, January 2009
