Case Report: Role of comprehensive rehabilitation In gaining recovery in a rare case of Polymyositis

Introduction

Idiopathic inflammatory myopathies include dermatomyositis (DM) and polymyositis (PM). Proximal muscular weakness, elevations in serum muscle enzymes, and, in the case of dermatomyositis, skin abnormalities identify them clinically. Immune pathways play an important role in the physiopathogenesis of polymyositis and dermatomyositis to varying degrees. Both diseases, as clinically diagnosed, have a general population prevalence rate of about 1 in 100,000.¹ The female to male ratio is roughly 2:1. Adults between the ages of 40 and 50 are most afflicted; however any age group could be affected. Polymyositis is significantly more prevalent in black people than in white people. Inflammatory infiltrates, particularly T-lymphocytes and macrophages, are prominent histology-based findings in muscle and cutaneous abnormalities distinguish dermatomyositis from polymyositis.²

Polymyositis affects the striated skeletal muscles but not the smooth muscles. Although the exact origin of PM is unknown, it has been postulated that some form of microvascular injury may trigger the synthesis of muscle auto antigens, which are then presented to T-lymphocytes by muscle macrophages. T-lymphocytes that have been activated proliferate and release cytokines such as interferon gamma and interleukin.³ Interferon gamma stimulates macrophages and causes the production of pro-inflammatory mediators such as IL-1 and tumour necrosis factor-alpha.⁴

The earliest signs are typically an asymptomatic deterioration of the pelvic and proximal lower leg muscles, making being able to walk, climb stairs, or getting from a chair difficult. Neck and shoulder girdle muscles are typically the first to be injured. The severity of the weakening might range from modest to near-paralysis. Weakness normally develops progressively over weeks to a period of time, although in rare circumstances, it may appear suddenly. Muscle weakness is the most typical indication of PM. The weakening is symmetric and affects both proximal limb muscles and neck flexors.⁵ Distal muscular weakness is unusual, but when it occurs, it should highlight the potential of another type of myopathy, such as inclusion body myositis. On occasion, PM patients may have muscle discomfort and tenderness, which may mimic the symptoms of polymyalgia rheumatica. Involvement of the oropharyngeal and upper esophageal striated muscles occurs in 10–15% of patients, has a poor prognosis, and can result in dysphagia, regurgitation, and aspiration pneumonia. Interstitial lung disease affects 5–10% of the population. Furthermore, because the diaphragm and intercostal muscles are involved, ventilatory failure may occur. Cardiac involvement is frequently asymptomatic.⁶

While there is no treatment for the condition, there has been research showing that medical treatment can reduce symptoms and give patients more control over their illness. Symptom management can help people perform better and live healthier lives. Polymyositis being idiopathic inflammatory myopathies cause a variety of systemic clinical symptoms. Among the most pertinent to the practice of physical therapy are muscle weakness, fatigue, and shortness of breath.⁷ The proximal musculature suffers the most, with considerable weakening throughout the neck, back, shoulders, forearms, thighs, and hips. Although distal weakness is possible, it is uncommon; however, the trunk is typically strong. Because the respiratory musculature is usually involved, the best evidence suggests combining resistance and aerobic exercise.⁸

Physical therapy helps PM patients maintain function and lower their risk of falling. It is critical that patients be active in order to retain function, and it is recommended that they exercise 5–6 times each week. Strengthening exercises should not be performed on consecutive days; instead, patients should take “active rest days,” during which they focus on Range of motion (ROM) posture, and relaxation rather than strengthening.⁹

Clinical findings

A physical examination was conducted after taking informed consent from the patient. The examination was divided into sensory and motor assessment. The sensations were intact. The motor assessment includes tone, reflex and strength assessment. The tone was normal. The reflexes were diminished (+) bilaterally as mentioned in Table 1.

Table 1. Assessment of deep tendon reflexes.

Reflex	Left	Right
Bicep Jerk	+	+
Tricep Jerk	+	+
Knee Jerk	+	+
Ankle Jerk	+	+

The strength assessment was done using Manual Muscle Testing Grading which revealed involvement of the proximal girdle (Table 2). The strength of the elbow and wrist, knee and ankle were 5/5.

Table 2. The Manual Muscle Testing Grading.

Joint	Muscles	Left	Right
Shoulder Joint	Flexors	3/5	3/5
	Extensors	3/5	3/5
	Abductors	3/5	3/5
	Adductors	4/5	4/5
	External Rotators	4/5	4/5
	Internal Rotators	4/5	4/5
Hip Joint	Flexors	3/5	3/5
	Extensors	3/5	3/5
	Abductors	4/5	4/5
	Adductors	4/5	4/5
	External Rotators	3/5	3/5
	Internal Rotators	3/5	3/5

There was tightness in the calf, hamstring and a positive Ober’s Test suggestive of iliotibial band tightness. There was hypermobility noted in ankle plantar flexors (Figure 1). There was difficulty in getting up from the floor (Figure 2).

Figure 1. Toes touching the plinth (hypermobility).

Figure 2. Difficulty in getting up from the floor.

Intervention

The interdisciplinary approach was used in managing this patient which was helpful in gaining early recovery. Physical therapy was focused to maintain function and lower their risk of falling. It is critical that the patient should be active in order to retain function, and she was recommended to exercise 5–6 times each week.

Strengthening exercises should not be performed on consecutive days; instead, patients should take “active rest days,” during which they focus on range of motion, posture, and relaxation rather than strengthening.

Muscle function preservation was done to prevent the disuse atrophy so mild resistance exercises were given. Strengthening of the distal muscles was initiated since it has the maximum chance for strength improvements, can significantly aid in a general improvement in activity of daily living function (Figure 3). Open chain exercises use less energy than closed chain workouts, but closed chain activities produce the best results in terms of functional mobility. Energy conservation techniques are essential (avoid high resistance open chain exercises and vigorous closed chain exercises). So adequate pacing was kept between the exercises. To prevent contracture, passive and active range of motion exercises (Figure 4) and stretching for calf, hamstrings and iliotibial band for three sets with 30 second hold each (Figure 5).

Figure 3. Strengthening using 0.5 kg weight.

Figure 4. Active range of motion exercise to maintain joint mobility.

Figure 5. Calf stretching to prevent contracture.

Discussion

Physical activity has been shown to improve health-related quality of life and well-being. Nonetheless, for a long time, it was widely assumed that physical activity could be hazardous to patients with myopathies, particularly inflammatory miopathies. The main concern for healthcare practitioners was that exercise could increase muscular inflammation in people with PM/DM, exacerbating muscle weakness.¹⁰

Symptoms vary considerably from patient to patient, and each drug has its own set of side effects. Because of the differences in symptom presentation and individual sensitivities to drugs, each patient’s medical therapy is unique. A combination of pharmacological treatment, physiotherapy, and alternative/holistic medicine may be used in treatment. Corticosteroids and immunosuppressant are often used in pharmacological management. Corticosteroids are used to decrease inflammation, ease pain, and increase strength, whereas immunosuppressant’s aid to minimize the body’s negative immunological response to the patient’s muscles.¹¹

Aerobic conditioning and vigorous resistance training are commonly used in the therapy of polymyositis and dermatomyositis patients. Aerobic exercise enhanced the performance of patients with PM/DM in the trials for which they were chosen, both by increasing maximal aerobic capacity and by boosting mitochondrial activity and oxygen uptake in skeletal muscles. After 6 months, an aerobic training programme resulted in a 28% rise in VO₂ max and a 12 week increase in CS (citrate synthase) and -HAD (β-hydroxyacyl-CoA dehydrogenase) activities. The analysed research concluded that concentric contractions were preferable for patients with PM/DM during aerobic performance. In reality, muscle fibres are stretched under eccentric contractions.¹²

Conclusion

Polymyositis is a potentially lethal condition that can exacerbate the symptoms of swallowing and breathing difficulties if left untreated. PM is more common in women, and the muscles that are most affected are the hips and thighs, upper arms, shoulder and neck. PM can also have an effect on the heart muscles, causing inflammatory myopathy, which is as well as the breathing muscles. Proximal muscular weakness is the most common clinical symptom. Extra muscular involvement may occur, including inflammatory arthritis, Reynaud’s phenomenon, myocarditis, and interstitial lung disease. During active illness, serum muscle enzymes (CK) are frequently increased. Auto antibodies of various types are frequently identified in the serum of PM patients. Electromyography and muscle MRI frequently reveal characteristic anomalies. Muscle biopsy is used to confirm the diagnosis. A steroid is typically used as the first-line treatment for PM. Prednisolone, in particular, is particularly successful at controlling inflammation and restoring swallowing, breathing, and hearing capabilities. To enhance movement and reduce pain, lifestyle changes such as drinking thicker fluids, eating softer or mashed foods, and engaging in light physiotherapy exercises should be implemented.

Consent

Written informed consent for publication of their clinical details and clinical images was obtained from the patient.