Polymyositis Clinical Presentation

Author: Ramesh Pappu, MD; Chief Editor: Herbert S Diamond, MD more...

Updated: Sep 30, 2011

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History
Physical Examination
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History

Symptoms of polymyositis gradually develop over a period of 3-6 months. Diagnosis is usually delayed, because, unlike in dermatomyositis, no associated rash occurs before the onset of muscle disease. Family history and medication history are important in excluding other causes of myopathy.

The history of patients with polymyositis or dermatomyositis typically includes the following:

Symmetrical, proximal muscle weakness with insidious onset
Muscles usually painless (Myalgias occur in fewer than 30% of patients.)
Dysphagia (30%) and aspiration, if pharyngeal and esophageal muscles are involved
Arthralgias may be associated
Difficulty kneeling, climbing or descending stairs, stepping onto a curb, raising arms, lifting objects, combing hair, and arising from a seated position
Weak neck extensors cause difficulty holding the head up
Involvement of pelvic girdle usually greater than upper body weakness
Cardiac involvement may cause symptoms of pericarditis or cardiomyopathy
Characteristic rash of face, trunk, and hands seen in dermatomyositis only

Muscular manifestations

Patients with polymyositis usually present with symmetrical, proximal muscle weakness in the upper and lower extremities. Weakness of neck flexors also occurs. Patients with polymyositis may report muscle pain and tenderness, which may be confused with symptoms of polymyalgia rheumatica. The disease may exist for several months before the patient seeks medical advice, and all of the muscles of the thighs, trunk, shoulders, hips, and upper arms are usually involved. Muscle weakness may fluctuate from week to week or from month to month.

Fine motor movements that depend on the strength of distal muscles, such as buttoning a shirt, sewing, knitting, or writing, are affected only late in the disease.

Dysphagia secondary to oropharyngeal and esophageal involvement occurs in about one third of patients with polymyositis and is a poor prognostic sign. Dysphonia is also a poor prognostic sign but is much less common.

Ocular muscles are never involved in generalized polymyositis. However, isolated orbital myositis, an inflammatory disorder involving the extraocular muscles, is well described. Facial and bulbar muscle weakness is extremely rare in individuals with polymyositis.

A family history of neuromuscular disease, endocrinopathy, or exposure to myotoxic drugs or toxins is absent.

Constitutional manifestations

Polymyositis is a systemic disease. Symptoms may include the following:

Morning stiffness
Fatigue
Anorexia
Fever (associated with antisynthetase antibodies such as anti-Jo-1)
Weight loss

Pulmonary manifestations

Pharyngeal and esophageal weakness may lead to aspiration pneumonia. Patients with polymyositis may experience exertional dyspnea secondary to weakness of chest wall muscles and diaphragmatic muscles. Patients receiving immunosuppressants are at an increased risk of infection.

Interstitial lung disease occurs in 5-30% of patients with idiopathic inflammatory myopathy (associated with antisynthetase antibodies, especially anti-Jo-1). Patients may be asymptomatic or present with exertional dyspnea, cough, and fever.^[6]

Interstitial pneumonitis, bronchiolitis obliterans organizing pneumonia, and pulmonary capillaritis have been described in conjunction with polymyositis.

Cardiac manifestations

Cardiac involvement is unusual and, if present, portends a bad prognosis. Rhythm disturbances, conduction defects, congestive heart failure, pericarditis, pulmonary hypertension, and myocarditis can occur.

Joint involvement

Patients can present with arthralgias or arthritis. Arthritis is usually symmetrical and involves the knees, wrists, and hands (associated with antisynthetase antibodies). A severe, deforming arthropathy without erosions has been reported; erosive changes are very rare.

Overlap syndromes

Polymyositis has been associated with other connective-tissue diseases, including the following:

Systemic lupus erythematosus
Rheumatoid arthritis
Mixed connective-tissue disease
Sjögren syndrome
Scleroderma

About 25% of patients with scleroderma have myositis; this phenomenon has been associated with anti-PM/Scl (anti-PM-1) antibody. In Japan, anti-Ku antibody has been described with this condition.

Gastrointestinal manifestations

Symptoms may include the following:

Dysphagia
Odynophagia
Nasal regurgitation
Reflux esophagitis
Abdominal bloating
Constipation

Renal manifestations

Intrinsic renal disease is rare in patients with polymyositis. Occasionally, severe rhabdomyolysis with myoglobinuria can result in acute tubular necrosis.

Cutaneous manifestations

Unlike in dermatomyositis, rash is absent in polymyositis. However, "mechanic's hands" (associated with antisynthetase antibodies), ie, hyperkeratotic eruptions over the finger pads and lateral aspects of the fingers, have been reported.

Raynaud phenomenon has been described in patients with antisynthetase antibodies. Rarely, periorbital edema may occur (best described in dermatomyositis). Calcinosis occurs in approximately 5% of patients with polymyositis (in association with sclerodermalike illness). Telangiectasias are uncommon.

Inclusion body myositis

This condition is a slowly progressive, idiopathic, inflammatory myopathy that mostly affects men older than age 50 years. Muscle involvement predominantly includes proximal muscles but may also include distal muscles (50%), and involvement may be asymmetrical. Dysphagia is found in most patients (60%).

Physical Examination

Nothing is characteristic about the muscle weakness in polymyositis. It is not painful, although a minority of patients report aches or cramps. On occasion, the muscle may be sore to palpation and may have a nodular and grainy feel.

Sensory examination findings are normal. Ocular muscles remain normal even in advanced, untreated cases. Facial muscles remain normal except in rare advanced cases.

The pharyngeal and neck flexor muscles are often involved, causing dysphagia and difficulty in holding up the head. When the patient is first seen, many of the muscles of the trunk, shoulders, hips, upper arms, and thighs are usually involved. In restricted forms of the disease, only the neck or paraspinal muscles (camptocormia) are affected.^[7]

In advanced cases and rarely in acute cases, respiratory muscles are affected. Severe weakness is almost always associated with muscular wasting. Dysphonia with nasal speech may be noted. Lung examination findings may include evidence of interstitial lung disease, such as dry inspiratory crackles in the lung bases ("Velcro").

The tendon reflexes are preserved, but they may be absent in severely weakened or atrophied muscles.

Primary cardiac abnormalities due to myocarditis may be present in a few patients. These abnormalities mainly manifest as atrioventricular conduction defects, tachyarrhythmias, low ejection fraction, dilated cardiomyopathy, or congestive heart failure.

General systemic disturbances, such as fever, malaise, weight loss, arthralgia, and Raynaud phenomenon, may occur when polymyositis is associated with a connective-tissue disorder.

Inclusion body myositis

Inclusion body myositis manifests as severe, proximal muscle weakness with atrophy, often with distal muscle weakness. The weakness may be asymmetrical. Deep tendon reflexes may be impaired or absent if weakness is severe.

Proceed to Differential Diagnoses

Contributor Information and Disclosures

Author

Ramesh Pappu, MD Adjunct Associate Professor of Medicine, Drexel University College of Medicine

Ramesh Pappu, MD is a member of the following medical societies: American Association of Physicians of Indian Origin and American College of Rheumatology

Disclosure: Nothing to disclose.

Coauthor(s)

Mythili Seetharaman, MD Consultant Rheumatologist, OAA, Allentown, PA; Clinical Assistant Professor, Thomas Jefferson University Hospital; Consulting Staff, Einstein Arthritis Center, Albert Einstein Medical Center, St Christopher's Hospital for Children

Mythili Seetharaman, MD is a member of the following medical societies: American College of Rheumatology and American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Additional Contributors

Michael S Beeson, MD, MBA, FACEP, Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine and Pharmacy; Attending Faculty, Akron General Medical Center

Michael S Beeson, MD, MBA, FACEP is a member of the following medical societies: American College of Emergency Physicians, Council of Emergency Medicine Residency Directors, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Thomas H Brannagan III, MD, Associate Professor of Clinical Neurology and Director, Peripheral Neuropathy Center, Columbia University, College of Physicians and Surgeons; Co-Director, EMG Laboratory, New York-Presbyterian Hospital, Columbia Campus, New York

Thomas H Brannagan III, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Peripheral Nerve Society

Disclosure: Nothing to disclose.

Lawrence H Brent, MD, Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

Zaineb Daud, MD, Consulting Staff, Department of Neurology, Medical College of Pennsylvania Hahnemann University

Disclosure: None

Gino A Farina, MD, FACEP, FAAEM, Associate Professor of Clinical Emergency Medicine, Albert Einstein College of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco de Assis Aquino Gondim, MD, MSc, PhD, Associate Professor of Neurology, Department of Neurology and Psychiatry, St Louis University School of Medicine

Francisco de Assis Aquino Gondim, MD, MSc, PhD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Movement Disorders Society

Disclosure: Nothing to disclose.

Aamir Hashmat, MD, Consulting Staff, Neurology and Neurodiagnostics Lab, Department of Neurology, Jeff Anderson Regional Medical Center

Aamir Hashmat, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society; American Medical Association, AO Foundation

Disclosure: None

Milind J Kothari, DO, Professor and Vice-Chair, Department of Neurology, Pennsylvania State University College of Medicine; Consulting Staff, Department of Neurology, Penn State Milton S Hershey Medical Center

Milind J Kothari, DO is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS, Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association

Disclosure: Nothing to disclose.

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Nicholas Lorenzo, MD, Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology

Disclosure: Nothing to disclose.

Henry Rosenkranz, MD, FAAEM, FACEP, Department of Emergency Medicine, Norwood Hospital

Henry Rosenkranz, MD, FAAEM, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Emergency Physicians

Disclosure: Nothing to disclose.

Erik D Schraga, MD, Staff Physician, Department of Emergency Medicine, Mills-Peninsula Emergency Medical Associates

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Additional Contributors

Michael S Beeson, MD, MBA, FACEP, Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine and Pharmacy; Attending Faculty, Akron General Medical Center

Disclosure: Nothing to disclose.

Zaineb Daud, MD, Consulting Staff, Department of Neurology, Medical College of Pennsylvania Hahnemann University

Disclosure: None

Disclosure: Nothing to disclose.

Francisco de Assis Aquino Gondim, MD, MSc, PhD, Associate Professor of Neurology, Department of Neurology and Psychiatry, St Louis University School of Medicine

Disclosure: Nothing to disclose.

Aamir Hashmat, MD, Consulting Staff, Neurology and Neurodiagnostics Lab, Department of Neurology, Jeff Anderson Regional Medical Center

Aamir Hashmat, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society; American Medical Association, AO Foundation

Disclosure: None

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association

Disclosure: Nothing to disclose.

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Nicholas Lorenzo, MD, Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology

Disclosure: Nothing to disclose.

Henry Rosenkranz, MD, FAAEM, FACEP, Department of Emergency Medicine, Norwood Hospital

Henry Rosenkranz, MD, FAAEM, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Emergency Physicians

Disclosure: Nothing to disclose.

Erik D Schraga, MD, Staff Physician, Department of Emergency Medicine, Mills-Peninsula Emergency Medical Associates

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

References

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Bronner IM, van der Meulen MF, de Visser M, Kalmijn S, van Venrooij WJ, Voskuyl AE, et al. Long-term outcome in polymyositis and dermatomyositis. Ann Rheum Dis. Nov 2006;65(11):1456-61. [Medline]. [Full Text].
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Cherin P, Pelletier S, Teixeira A, et al. Results and long-term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients. Arthritis Rheum. Feb 2002;46(2):467-74. [Medline].
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Hengstman G, Van den Housen F, Van Engelen B. Anti-TNF-blockade with infliximab (Remicade) in polymyositis and dermatomyositis. Arthritis and Rheumatology. 2000;43(9):S193.
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MRI of thighs showing increased signal in the quadriceps muscles bilaterally consistent with inflammatory myositis.

Histopathology of polymyositis showing endomysial mononuclear inflammatory infiltrate and muscle fiber necrosis.

Close view of muscle biopsy, showing chronic inflammatory infiltrate consisting of T lymphocytes, especially CD8+ T lymphocytes.

Hematoxylin and eosin frozen section shows polymyositis. Endomysial chronic inflammation is present among intact myofibers, which are remarkable only for increased variability of fiber size. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains an abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of the muscle is present in the lower left quadrant of the photomicrograph. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on a nonnecrotic myofiber by autoaggressive T lymphocytes. On the left, the central myofiber is intact. On the right, it is obliterated by a segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include an admixture of CD8 T lymphocytes and macrophages in the inflammatory process. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in the center has a mild perivascular chronic inflammatory infiltrate. The endothelium is plump. The wall is not necrotic. A few lymphocytes in the wall of the vessel are probably in transit from the lumen to the external aspect of the vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin section shows polymyositis. Longitudinal section shows a dense, chronic, endomysial inflammatory infiltrate. Image courtesy of Roberta J. Seidman, MD.

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