Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy Saleh R. Al-Ballaa, FRCPC; Abdullah N. Al-Dalaan, ABIM, FACR; Kamal M. El-Ramahi, MD; Mansour A. Al-Janadi, FRCPC; Ahmed Al-Shaikh, ABIM; Sultan Bahabri, FRCP(C) From the College of Medicine (Drs. Al-Ballaa and Al-Janadi), King Saud University and Department of Medicine (Drs. Al-Dalaan, El-Ramahi, Al-Shaik, and Bahabri), King Faisal Specialist Hospital and Research Centre, Riyadh. Address reprint requests and correspondence to Dr. Al-Dalaan: Consultant Rheumatologist, Department of Medicine and Deputy Director, Academic Affairs and Postgraduate Education, King Faisal Specialist Hospital and Research Centre, P.O. Box 3354, Riyadh 11211, Saudi Arabia. Accepted for publication 8 March 1993. A retrospective study of 22 adult patients with dermatomyositis (DM) or polymyositis (PM) was performed. Male to female ratio was 1:2.7. Mean age of onset was 37.3 ± (16.3) and symptoms were present for a mean of 11.2 ± 14.6 months before diagnosis. Primary polymyositis was diagnosed in 11 (50%), primary dermatomyositis in three (13.6%). PM/DM was associated with connective tissue disease in three (13.6%) and malignancy in five patients (22.7%). Muscle disease followed the diagnosis of malignancy by a mean of 12.2 months (one to 36 months). All were female. Diffuse erythema was observed in all three patients with DM and malignancy. Arthritis was seen more frequently in our patients (55%). Sixty-eight percent of patients showed substantial improvement of muscle disease with steroids alone or in combination with other immunosuppressive agents, 18% did not improve or their disease progressed in spite of the treatment. Three patients died (14%), two from respiratory failure and one from underlying malignancy. Ann Saudi Med 1993;13(6):525-529. SR Al-Ballaa, AN Al-Dalaan, KM El-Ramahi, MA Al-Janadi, A Al-Shaikh, S Bahabri, Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy. 1993; 13(6): 525-529 Polymyositis (PM) is an inflammatory disease of striated muscles of unknown etiology [1,2]. The disease is characterized by the presence of inflammatory infiltrates in the skeletal muscle and associated with muscle fiber necrosis and/or degeneration [3-5]. When accompanied by typical skin changes, it is called dermatomyositis (DM) [1,6,7]. The disease has been classified into five types [1]. The most widely accepted diagnostic criteria are those of Bohan and Peter [1]. The incidence of the disease varies between one to eight cases/million population/year with a prevalence rate of 10.4 to 63/million [8,10]. Distribution of cases among various subgroups and severity of the disease vary among different series. Different referral patterns have been cited as a possible explanation [11,12]. In this report, we present the clinical features, laboratory findings and outcome of 22 adult patients with PM/DM seen in King Khalid University Hospital (KKUH) and King Faisal Specialist Hospital and Research Centre (KFSH&RC). Patients and Methods The medical records of 34 patients with diagnosis of PM or DM in contributing hospitals were reviewed. One patient with RA and concomitant hypothyroidism presenting with muscle weakness and raised muscle enzyme [13] and 11 patients with childhood onset (before age 16) were excluded. The remaining 22 patients fulfilled the proposed criteria for diagnosis of PM/DM (symmetrical muscle reactive, raised muscle enzymes, positive muscle biopsy, electromyographic abnormality consistent with myositis and dermatologie features). Furthermore, the disease was classified as definite DM (three or four criteria plus the rash), definite PM (four criteria), probable DM (two criteria plus the rash), probable PM (three criteria), probable DM (one criterion plus the rash), and possible PM (two criteria) [1]. The British Medical Research Council method to score muscle power was used as follows: 0 = no contraction; 1 = flicker or trace contraction; 2 = active movement but not against gravity; 3 = active movement against gravity; 4 = active movement against gravity and resistance; 5 = normal power 17]. All patients had electromyograph (EMG) studies while only 21 had muscle biopsy. The following laboratory studies were done in most patients; complete blood count (CBC), erythrocyte sedimentation rate (ESR), creatinine kinase (CK), lactic dehydrogenase (LDH), aspartate aminotransferase (AST), alanine aminotransferase (ALT), antinuclear antibodies (ANA), rheumatoid factor (RF) and antibodies to extractable nuclear antigen (anti-ENA). Patients were classified further into four subgroups: Group 1, primary PM; Group 2, primary DM; Group 3, PM/DM associated with malignancy and Group 4, PM/DM associated with a connective tissue disease. In the latter
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Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with MalignancyPattern of Adult Onset of Polymyositis and Dermatomyositis and Association with
Malignancy
Kamal M. El-Ramahi, MD; Mansour A. Al-Janadi, FRCPC;
Ahmed Al-Shaikh, ABIM; Sultan Bahabri, FRCP(C)
From the College of Medicine (Drs. Al-Ballaa and Al-Janadi), King Saud University and Department of Medicine (Drs. Al-Dalaan, El-Ramahi, Al-Shaik,
and Bahabri), King Faisal Specialist Hospital and Research Centre, Riyadh.
Address reprint requests and correspondence to Dr. Al-Dalaan: Consultant Rheumatologist, Department of Medicine and Deputy Director, Academic
Affairs and Postgraduate Education, King Faisal Specialist Hospital and Research Centre, P.O. Box 3354, Riyadh 11211, Saudi Arabia.
Accepted for publication 8 March 1993.
A retrospective study of 22 adult patients with dermatomyositis (DM) or polymyositis (PM) was performed. Male to
female ratio was 1:2.7. Mean age of onset was 37.3 ± (16.3) and symptoms were present for a mean of 11.2 ± 14.6
months before diagnosis. Primary polymyositis was diagnosed in 11 (50%), primary dermatomyositis in three
(13.6%). PM/DM was associated with connective tissue disease in three (13.6%) and malignancy in five patients
(22.7%). Muscle disease followed the diagnosis of malignancy by a mean of 12.2 months (one to 36 months). All
were female. Diffuse erythema was observed in all three patients with DM and malignancy. Arthritis was seen more
frequently in our patients (55%). Sixty-eight percent of patients showed substantial improvement of muscle disease
with steroids alone or in combination with other immunosuppressive agents, 18% did not improve or their disease
progressed in spite of the treatment. Three patients died (14%), two from respiratory failure and one from underlying
malignancy. Ann Saudi Med 1993;13(6):525-529.
SR Al-Ballaa, AN Al-Dalaan, KM El-Ramahi, MA Al-Janadi, A Al-Shaikh, S Bahabri, Pattern of Adult Onset of
Polymyositis and Dermatomyositis and Association with Malignancy. 1993; 13(6): 525-529
Polymyositis (PM) is an inflammatory disease of striated muscles of unknown etiology [1,2]. The disease is
characterized by the presence of inflammatory infiltrates in the skeletal muscle and associated with muscle fiber
necrosis and/or degeneration [3-5]. When accompanied by typical skin changes, it is called dermatomyositis (DM)
[1,6,7]. The disease has been classified into five types [1]. The most widely accepted diagnostic criteria are those of
Bohan and Peter [1]. The incidence of the disease varies between one to eight cases/million population/year with a
prevalence rate of 10.4 to 63/million [8,10]. Distribution of cases among various subgroups and severity of the
disease vary among different series. Different referral patterns have been cited as a possible explanation [11,12].
In this report, we present the clinical features, laboratory findings and outcome of 22 adult patients with
PM/DM seen in King Khalid University Hospital (KKUH) and King Faisal Specialist Hospital and Research Centre
(KFSH&RC).
Patients and Methods
The medical records of 34 patients with diagnosis of PM or DM in contributing hospitals were reviewed. One
patient with RA and concomitant hypothyroidism presenting with muscle weakness and raised muscle enzyme [13]
and 11 patients with childhood onset (before age 16) were excluded. The remaining 22 patients fulfilled the
proposed criteria for diagnosis of PM/DM (symmetrical muscle reactive, raised muscle enzymes, positive muscle
biopsy, electromyographic abnormality consistent with myositis and dermatologie features). Furthermore, the
disease was classified as definite DM (three or four criteria plus the rash), definite PM (four criteria), probable DM
(two criteria plus the rash), probable PM (three criteria), probable DM (one criterion plus the rash), and possible PM
(two criteria) [1]. The British Medical Research Council method to score muscle power was used as follows: 0 = no
contraction; 1 = flicker or trace contraction; 2 = active movement but not against gravity; 3 = active movement
against gravity; 4 = active movement against gravity and resistance; 5 = normal power 17]. All patients had
electromyograph (EMG) studies while only 21 had muscle biopsy. The following laboratory studies were done in
most patients; complete blood count (CBC), erythrocyte sedimentation rate (ESR), creatinine kinase (CK), lactic
dehydrogenase (LDH), aspartate aminotransferase (AST), alanine aminotransferase (ALT), antinuclear antibodies
(ANA), rheumatoid factor (RF) and antibodies to extractable nuclear antigen (anti-ENA).
Patients were classified further into four subgroups: Group 1, primary PM; Group 2, primary DM; Group 3,
PM/DM associated with malignancy and Group 4, PM/DM associated with a connective tissue disease. In the latter
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
group, patients were required to satisfy independent classification criteria for systemic lupus erythematosus (SLE)
[14], systemic sclerosis [15] and/or Sjogren syndrome [16].
Results
Of the 22 patients, 16 were females and six were males (female to male ratio of 2.7:1). The mean age is 39.7±16
(range 17 to 70). Their mean age of onset was 37.3±16.3.
Classification is shown in Table 1;. The diagnosis of polymyositis was categorized as definite in nine patients
and probable in six while only six patients were classified as definite and one patient as probable dermatomyositis
(Bohan and Peter criteria) [1]. Clinical findings are summarized in Tables 2 and 3. Muscle weakness was observed
in all patients while muscle pain and tenderness were observed in 14 patients. Arthralgia was reported in 17 patients
(77.3). Arthritis occurred in 12 patients. The most frequent joints involved were knees followed by small joints of
the hand (Table 2).
Fever occurred in nine patients during the course of their illness. Fifteen patients had weight loss. Dysphagia
was reported in nine patients (40.9%). Shortness of breath and cough occurred in five and four patients, respectively
(Table 3).
Laboratory findings are summarized in Table 4. The ESR was normal in 10 patients. CK was elevated in 20
patients, 4298±6507 (range 33 to 25,256). With treatment, CK decreased to 1137±3781 (range 20 to 16,648). In
12/18 patients, it returned to normal and remained marginally elevated in three patients.
Table 1. Classification of the patients.
Classification
PM/DM with CT dis (2PM/DM) 3 (13.6%)
PM=polymyositis; DM=dermatomyositis.
Table 2. Muscle and skin involvement of patients with PM/DM.
Clinical Feature No. of Patients (%)
Muscle weakness 22 (100)
Muscle pain 14 (63.6)
- Heliotrope 5
Age of onset 37.3 ± 16.3
Sex (M/F) 6/16 (1:2.7)
Heart failure 2 ( 9.1%)
Conduction deficit 1 ( 4.5%)
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
Table 4. Laboratory findings in patient with PM-DM.
Laboratory
ANA 13/22 59
RF 8/21 38
ENA 4/14 29
AST=aspartate aminotransferase; ANA=antinuclear antibodies; RF=rheumatoid factor;
ENA=extractable nuclear antigen.
ANA by immunofluorescence was positive in 13 patients (59.1%), titer was ranging from 1/40 to 1/10,240, anti-
ENA by ELIAS was positive in four out of 14 patients tested (28.6%). None reached a diagnostic level of mixed
connective tissue disease. RF by ELISA was positive in eight out of 21 patients (38.1%), range was 1/40 to 1/320.
All patients included in this study had an EMG. It was normal in three patients; in the rest of the patients, it
showed features considered to be compatible with PM, suggestive of polymyositis in five patients, and diagnostic in
14 patients. Muscle biopsy and routine panel staining was performed on 21 patients. Muscle biopsy was normal in
four patients and diagnostic in 14 patients. In three patients, no active necrosis, phagocytosis or perivascular atrophy
were seen, and the biopsy was reported as compatible with PM.
Prednisone (1 to 2.5 mg/kg) was used in 21 patients as the initial therapy; 15 patients showed substantial
improvement on therapy as judged by increase in muscle power (Figure) and decrease in muscle enzymes. Two
cases remained the same, both of which were thought to represent advanced inactive disease. In eight patients,
methotrexate or azathioprine was used in addition to steroids. In two patients, the disease progressed despite therapy.
A total of three patients died; two from respiratory failure and one from underlying malignancy.
The clinical features and demographic data of patients with malignancy and DM/PM are shown in Table 5. All
patients were female. Their mean age of onset was 51.6±14.4Y compared to 33.1 ± 15.2 for patients without
malignancy (P <0.02). All patients with malignancy had raised CK and ANA was positive in four patients, muscle
biopsies were interpreted as compatible with polymyositis in four patients and normal in one patient. Diffuse
erythema was observed in all three patients with DM and malignancy.
Discussion
The mean age and sex ratio of patients in this series is similar to other reports [2, 11, 12, 18]. The proportion of
patients with primary dermatomyositis (13.6%) is less than that observed by Hochberg et al (28%) [11], Hoffman et
al (22%) [12] or Ramirez et al (68%) [18]. This may reflect racial differences as patients in these series were
Caucasian while most of our patients were non-Caucasian. Darker color makes it difficult to observe the typical rash
of dermatomyositis. Overall, 14 (63.6%) patients were characterized as definite PM/DM and eight (36.4%) as
probable PM/DM using the criteria of Bohan and Peter [1]. However, all patients in Group 2 were categorized as
definite disease, which is simlar to that of Hochberg et al [11].
The frequency of malignancy among adult patients with PM/DM varies from 7% to 24% [11,12,19]. This
association is more maintained in patients with DM. Patients tend to be older than patients with PM/DM without
malignancy. Associated malignancy was diagnosed in five patients (22.7%) in this series. Of these, three had
dermatomyositis and two had polymyositis. The influence of referral pattern on the proportion of patients with
malignancy and PM/DM is evident from the findings that four of 12 patients (33.3%) reported from KFSH&RC (a
tertiary referral and oncology center for the area) had malignancy compared to 10% of patients from the KKUH.
The extent of investigation to look for malignancy in patients with PM/DM is still unsettled [20,22]. However,
this should be guided by the history and physical examination [21,22]. A complete annual physical examination
including pelvic and rectal examination, urinalysis, complete blood count, blood chemistry tests and chest x-ray
should be adequate [21,23]. Arthritis was found in a much higher proportion in our patients than other series
[11,12,18]. It was observed in. Group 1 (56%), Group 2 (30%) and in patients with myositis and malignancy (Group
3), where three out of five patients (60%) had arthritis.
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
Figure: Initial presentation of muscle weakness of all patients and response to therapy.
Table 5. Clinical and laboratory data on PM/DM patients with malignancy.
The reason for this observation is not clear. It is unlikely that patients with overlap groups (Group 4) were
overlooked and included in other groups, resulting in the higher proportion of patients with arthritis found in this
study since arthritis was noted in 60% of patients with Group 3, PM/DM.
Acknowledgment
The secretarial assistance of Misses Aleli Sobingsobing and Mabeth Martinez is greatly appreciated.
References
1. Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975;292:344-407.
2. Bohan A, Peter JN, Bowman RL, et al. A computer assisted analysis of 153 paitents with polymyositis and dermatomyositis.
Med 1977;56:255-86.
3. Plotz PH, Dalakas M, Leff RL, et al. Current concepts in the idiopathic inflammatory myopathies: polymyositis,
dermatomyositis and related disorders. Ann Int Med 1989;111:143-57.
4. Targof IN, Reichlin M. Immunological aspects in Mastalgia FL, ed. Inflammatory disease of muscle. Oxford, UK, Blackwell
Scientific 1988;37-70.
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
5. Pedro-Botet JC, Grau JM, Casademont J, et al. Characterization of mononuclear exudates in idiopathic inflammatory
myopathies. Virchows Archiv A Pathol Anat Histopathol, 1988,412:371-4.
6. Ansell BM. Inflammatory disease of muscle. Clin Rheum Dis 1984;10:1-215.
7. Banker BQ, Engel AG. The polymyositis and dermatomyositis syndromes. In: Myology, Engel AG, Banker BQ, eds. New
York:McGraw Hill, 1986;1:385-422.
8. Medsger TA, Dawson WN, Masi AT. The epidemiology of polymyositis. Am J Med 1979;48:715-23.
9. Kurland LT, Hauser WA, Ferguson RH, et al. Epidemiologie features of diffuse connective tissue disorders in Rochester,
Minnesota, USA 1951-1967 with special reference to systemic lupus erythematosus. Mayo Clin Proc, 1969;44:649-63.
10. Oddis CV, Conte C, Casterline GZ, et al. Incidence of polymyositis-dermatomyositis (PM/DM): a twenty year survey of
hospital diagnosed cases. Arthritis 1988, Rheum 31(suppl):557.
11. Hochberg MC, Feldman D, Stevens MB. Adult onset polymyositis/dermatomyositis, an analysis of clinical and laboratory
features and survival in 76 patients with a review of the literature. Semin Arthritis Rheum 1986;15:168-78.
12. Hoffman GS, Franck WA, Raddatz DA, Stallones L. Presentation, treatment and prognosis of idiopathic inflammatory muscle
disease in a rural hospital. Am J Med 1983;75:433-8.
13. Hochberg, MC, Koppes GM, Edwards CQ, et al. Hypothyroidism presenting as a polymyositis-like syndrome: report of two
cases. Arthritis Rheum 1976;19:1363:66.
14. Cohen AS, Reynolds WE, Franklin FC, et al. Preliminary criteria for the classification of systemic lupus erythematosus. Bull
Rheum Dis 1971;21:643-8.
15. Masi AT, Medsger TA, Rodnan GP, et al. Methods and preliminary results of the scleroderma criteria cooperative study of
the American Rheumatism Association. Clin Rheum Dis 1979;5:27-48.
16. Strand V, Talal N. Advances in the diagnosis and concept of Sjogren syndrome (autoimmune exocrinopathy). Bull Rheum
Dis 1980;30:1046-52.
17. Riddoch G, Bristow WR, Cairns HWB, et al. Aids to the investigation of peripheral nerve injuries. Memorandum. Med Res
Council 1943;7:1-17.
18. Ramirez G, Asherson RA, Khamashta MA, et al. Adult onset polymyositis-dermatomyositis description of 25 patients with
emphasis on treatment. Semin in Arthritis Rheum 1990;20:114-20.
19. Callen JP, Hyla JF, Bole GG, et al. The relationship of dermatomyositis and polymyositis to internal malignancy. Arch Derm
1980;116:295-8.
20. Lakhanpal S, Bunch TW, Ilstrup DM, et al. Polymyositis-dermatomyositis and malignant lesions: does an association exist?
Mayo Clin Proc 1980;616:45-53.
21. Callen JP. The value of malignancy evaluation in patients with dermatomyositis. J Am Acad Dermatol 1982;6:163-9.
22. Richardson JB, Callen JP. Dermatomyositis and malignancy. Med Clin North Am 1989;73:1211-20.
23. Dalakas MC. Polymyositis, dermatomyositis and inclusion-body myositis. N Engl J Med 1991;323:1487-1508.
Malignancy
Kamal M. El-Ramahi, MD; Mansour A. Al-Janadi, FRCPC;
Ahmed Al-Shaikh, ABIM; Sultan Bahabri, FRCP(C)
From the College of Medicine (Drs. Al-Ballaa and Al-Janadi), King Saud University and Department of Medicine (Drs. Al-Dalaan, El-Ramahi, Al-Shaik,
and Bahabri), King Faisal Specialist Hospital and Research Centre, Riyadh.
Address reprint requests and correspondence to Dr. Al-Dalaan: Consultant Rheumatologist, Department of Medicine and Deputy Director, Academic
Affairs and Postgraduate Education, King Faisal Specialist Hospital and Research Centre, P.O. Box 3354, Riyadh 11211, Saudi Arabia.
Accepted for publication 8 March 1993.
A retrospective study of 22 adult patients with dermatomyositis (DM) or polymyositis (PM) was performed. Male to
female ratio was 1:2.7. Mean age of onset was 37.3 ± (16.3) and symptoms were present for a mean of 11.2 ± 14.6
months before diagnosis. Primary polymyositis was diagnosed in 11 (50%), primary dermatomyositis in three
(13.6%). PM/DM was associated with connective tissue disease in three (13.6%) and malignancy in five patients
(22.7%). Muscle disease followed the diagnosis of malignancy by a mean of 12.2 months (one to 36 months). All
were female. Diffuse erythema was observed in all three patients with DM and malignancy. Arthritis was seen more
frequently in our patients (55%). Sixty-eight percent of patients showed substantial improvement of muscle disease
with steroids alone or in combination with other immunosuppressive agents, 18% did not improve or their disease
progressed in spite of the treatment. Three patients died (14%), two from respiratory failure and one from underlying
malignancy. Ann Saudi Med 1993;13(6):525-529.
SR Al-Ballaa, AN Al-Dalaan, KM El-Ramahi, MA Al-Janadi, A Al-Shaikh, S Bahabri, Pattern of Adult Onset of
Polymyositis and Dermatomyositis and Association with Malignancy. 1993; 13(6): 525-529
Polymyositis (PM) is an inflammatory disease of striated muscles of unknown etiology [1,2]. The disease is
characterized by the presence of inflammatory infiltrates in the skeletal muscle and associated with muscle fiber
necrosis and/or degeneration [3-5]. When accompanied by typical skin changes, it is called dermatomyositis (DM)
[1,6,7]. The disease has been classified into five types [1]. The most widely accepted diagnostic criteria are those of
Bohan and Peter [1]. The incidence of the disease varies between one to eight cases/million population/year with a
prevalence rate of 10.4 to 63/million [8,10]. Distribution of cases among various subgroups and severity of the
disease vary among different series. Different referral patterns have been cited as a possible explanation [11,12].
In this report, we present the clinical features, laboratory findings and outcome of 22 adult patients with
PM/DM seen in King Khalid University Hospital (KKUH) and King Faisal Specialist Hospital and Research Centre
(KFSH&RC).
Patients and Methods
The medical records of 34 patients with diagnosis of PM or DM in contributing hospitals were reviewed. One
patient with RA and concomitant hypothyroidism presenting with muscle weakness and raised muscle enzyme [13]
and 11 patients with childhood onset (before age 16) were excluded. The remaining 22 patients fulfilled the
proposed criteria for diagnosis of PM/DM (symmetrical muscle reactive, raised muscle enzymes, positive muscle
biopsy, electromyographic abnormality consistent with myositis and dermatologie features). Furthermore, the
disease was classified as definite DM (three or four criteria plus the rash), definite PM (four criteria), probable DM
(two criteria plus the rash), probable PM (three criteria), probable DM (one criterion plus the rash), and possible PM
(two criteria) [1]. The British Medical Research Council method to score muscle power was used as follows: 0 = no
contraction; 1 = flicker or trace contraction; 2 = active movement but not against gravity; 3 = active movement
against gravity; 4 = active movement against gravity and resistance; 5 = normal power 17]. All patients had
electromyograph (EMG) studies while only 21 had muscle biopsy. The following laboratory studies were done in
most patients; complete blood count (CBC), erythrocyte sedimentation rate (ESR), creatinine kinase (CK), lactic
dehydrogenase (LDH), aspartate aminotransferase (AST), alanine aminotransferase (ALT), antinuclear antibodies
(ANA), rheumatoid factor (RF) and antibodies to extractable nuclear antigen (anti-ENA).
Patients were classified further into four subgroups: Group 1, primary PM; Group 2, primary DM; Group 3,
PM/DM associated with malignancy and Group 4, PM/DM associated with a connective tissue disease. In the latter
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
group, patients were required to satisfy independent classification criteria for systemic lupus erythematosus (SLE)
[14], systemic sclerosis [15] and/or Sjogren syndrome [16].
Results
Of the 22 patients, 16 were females and six were males (female to male ratio of 2.7:1). The mean age is 39.7±16
(range 17 to 70). Their mean age of onset was 37.3±16.3.
Classification is shown in Table 1;. The diagnosis of polymyositis was categorized as definite in nine patients
and probable in six while only six patients were classified as definite and one patient as probable dermatomyositis
(Bohan and Peter criteria) [1]. Clinical findings are summarized in Tables 2 and 3. Muscle weakness was observed
in all patients while muscle pain and tenderness were observed in 14 patients. Arthralgia was reported in 17 patients
(77.3). Arthritis occurred in 12 patients. The most frequent joints involved were knees followed by small joints of
the hand (Table 2).
Fever occurred in nine patients during the course of their illness. Fifteen patients had weight loss. Dysphagia
was reported in nine patients (40.9%). Shortness of breath and cough occurred in five and four patients, respectively
(Table 3).
Laboratory findings are summarized in Table 4. The ESR was normal in 10 patients. CK was elevated in 20
patients, 4298±6507 (range 33 to 25,256). With treatment, CK decreased to 1137±3781 (range 20 to 16,648). In
12/18 patients, it returned to normal and remained marginally elevated in three patients.
Table 1. Classification of the patients.
Classification
PM/DM with CT dis (2PM/DM) 3 (13.6%)
PM=polymyositis; DM=dermatomyositis.
Table 2. Muscle and skin involvement of patients with PM/DM.
Clinical Feature No. of Patients (%)
Muscle weakness 22 (100)
Muscle pain 14 (63.6)
- Heliotrope 5
Age of onset 37.3 ± 16.3
Sex (M/F) 6/16 (1:2.7)
Heart failure 2 ( 9.1%)
Conduction deficit 1 ( 4.5%)
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
Table 4. Laboratory findings in patient with PM-DM.
Laboratory
ANA 13/22 59
RF 8/21 38
ENA 4/14 29
AST=aspartate aminotransferase; ANA=antinuclear antibodies; RF=rheumatoid factor;
ENA=extractable nuclear antigen.
ANA by immunofluorescence was positive in 13 patients (59.1%), titer was ranging from 1/40 to 1/10,240, anti-
ENA by ELIAS was positive in four out of 14 patients tested (28.6%). None reached a diagnostic level of mixed
connective tissue disease. RF by ELISA was positive in eight out of 21 patients (38.1%), range was 1/40 to 1/320.
All patients included in this study had an EMG. It was normal in three patients; in the rest of the patients, it
showed features considered to be compatible with PM, suggestive of polymyositis in five patients, and diagnostic in
14 patients. Muscle biopsy and routine panel staining was performed on 21 patients. Muscle biopsy was normal in
four patients and diagnostic in 14 patients. In three patients, no active necrosis, phagocytosis or perivascular atrophy
were seen, and the biopsy was reported as compatible with PM.
Prednisone (1 to 2.5 mg/kg) was used in 21 patients as the initial therapy; 15 patients showed substantial
improvement on therapy as judged by increase in muscle power (Figure) and decrease in muscle enzymes. Two
cases remained the same, both of which were thought to represent advanced inactive disease. In eight patients,
methotrexate or azathioprine was used in addition to steroids. In two patients, the disease progressed despite therapy.
A total of three patients died; two from respiratory failure and one from underlying malignancy.
The clinical features and demographic data of patients with malignancy and DM/PM are shown in Table 5. All
patients were female. Their mean age of onset was 51.6±14.4Y compared to 33.1 ± 15.2 for patients without
malignancy (P <0.02). All patients with malignancy had raised CK and ANA was positive in four patients, muscle
biopsies were interpreted as compatible with polymyositis in four patients and normal in one patient. Diffuse
erythema was observed in all three patients with DM and malignancy.
Discussion
The mean age and sex ratio of patients in this series is similar to other reports [2, 11, 12, 18]. The proportion of
patients with primary dermatomyositis (13.6%) is less than that observed by Hochberg et al (28%) [11], Hoffman et
al (22%) [12] or Ramirez et al (68%) [18]. This may reflect racial differences as patients in these series were
Caucasian while most of our patients were non-Caucasian. Darker color makes it difficult to observe the typical rash
of dermatomyositis. Overall, 14 (63.6%) patients were characterized as definite PM/DM and eight (36.4%) as
probable PM/DM using the criteria of Bohan and Peter [1]. However, all patients in Group 2 were categorized as
definite disease, which is simlar to that of Hochberg et al [11].
The frequency of malignancy among adult patients with PM/DM varies from 7% to 24% [11,12,19]. This
association is more maintained in patients with DM. Patients tend to be older than patients with PM/DM without
malignancy. Associated malignancy was diagnosed in five patients (22.7%) in this series. Of these, three had
dermatomyositis and two had polymyositis. The influence of referral pattern on the proportion of patients with
malignancy and PM/DM is evident from the findings that four of 12 patients (33.3%) reported from KFSH&RC (a
tertiary referral and oncology center for the area) had malignancy compared to 10% of patients from the KKUH.
The extent of investigation to look for malignancy in patients with PM/DM is still unsettled [20,22]. However,
this should be guided by the history and physical examination [21,22]. A complete annual physical examination
including pelvic and rectal examination, urinalysis, complete blood count, blood chemistry tests and chest x-ray
should be adequate [21,23]. Arthritis was found in a much higher proportion in our patients than other series
[11,12,18]. It was observed in. Group 1 (56%), Group 2 (30%) and in patients with myositis and malignancy (Group
3), where three out of five patients (60%) had arthritis.
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
Figure: Initial presentation of muscle weakness of all patients and response to therapy.
Table 5. Clinical and laboratory data on PM/DM patients with malignancy.
The reason for this observation is not clear. It is unlikely that patients with overlap groups (Group 4) were
overlooked and included in other groups, resulting in the higher proportion of patients with arthritis found in this
study since arthritis was noted in 60% of patients with Group 3, PM/DM.
Acknowledgment
The secretarial assistance of Misses Aleli Sobingsobing and Mabeth Martinez is greatly appreciated.
References
1. Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975;292:344-407.
2. Bohan A, Peter JN, Bowman RL, et al. A computer assisted analysis of 153 paitents with polymyositis and dermatomyositis.
Med 1977;56:255-86.
3. Plotz PH, Dalakas M, Leff RL, et al. Current concepts in the idiopathic inflammatory myopathies: polymyositis,
dermatomyositis and related disorders. Ann Int Med 1989;111:143-57.
4. Targof IN, Reichlin M. Immunological aspects in Mastalgia FL, ed. Inflammatory disease of muscle. Oxford, UK, Blackwell
Scientific 1988;37-70.
Pattern of Adult Onset of Polymyositis and Dermatomyositis and Association with Malignancy
Annals of Saudi Medicine, Vol 13 No. 6; 1993
5. Pedro-Botet JC, Grau JM, Casademont J, et al. Characterization of mononuclear exudates in idiopathic inflammatory
myopathies. Virchows Archiv A Pathol Anat Histopathol, 1988,412:371-4.
6. Ansell BM. Inflammatory disease of muscle. Clin Rheum Dis 1984;10:1-215.
7. Banker BQ, Engel AG. The polymyositis and dermatomyositis syndromes. In: Myology, Engel AG, Banker BQ, eds. New
York:McGraw Hill, 1986;1:385-422.
8. Medsger TA, Dawson WN, Masi AT. The epidemiology of polymyositis. Am J Med 1979;48:715-23.
9. Kurland LT, Hauser WA, Ferguson RH, et al. Epidemiologie features of diffuse connective tissue disorders in Rochester,
Minnesota, USA 1951-1967 with special reference to systemic lupus erythematosus. Mayo Clin Proc, 1969;44:649-63.
10. Oddis CV, Conte C, Casterline GZ, et al. Incidence of polymyositis-dermatomyositis (PM/DM): a twenty year survey of
hospital diagnosed cases. Arthritis 1988, Rheum 31(suppl):557.
11. Hochberg MC, Feldman D, Stevens MB. Adult onset polymyositis/dermatomyositis, an analysis of clinical and laboratory
features and survival in 76 patients with a review of the literature. Semin Arthritis Rheum 1986;15:168-78.
12. Hoffman GS, Franck WA, Raddatz DA, Stallones L. Presentation, treatment and prognosis of idiopathic inflammatory muscle
disease in a rural hospital. Am J Med 1983;75:433-8.
13. Hochberg, MC, Koppes GM, Edwards CQ, et al. Hypothyroidism presenting as a polymyositis-like syndrome: report of two
cases. Arthritis Rheum 1976;19:1363:66.
14. Cohen AS, Reynolds WE, Franklin FC, et al. Preliminary criteria for the classification of systemic lupus erythematosus. Bull
Rheum Dis 1971;21:643-8.
15. Masi AT, Medsger TA, Rodnan GP, et al. Methods and preliminary results of the scleroderma criteria cooperative study of
the American Rheumatism Association. Clin Rheum Dis 1979;5:27-48.
16. Strand V, Talal N. Advances in the diagnosis and concept of Sjogren syndrome (autoimmune exocrinopathy). Bull Rheum
Dis 1980;30:1046-52.
17. Riddoch G, Bristow WR, Cairns HWB, et al. Aids to the investigation of peripheral nerve injuries. Memorandum. Med Res
Council 1943;7:1-17.
18. Ramirez G, Asherson RA, Khamashta MA, et al. Adult onset polymyositis-dermatomyositis description of 25 patients with
emphasis on treatment. Semin in Arthritis Rheum 1990;20:114-20.
19. Callen JP, Hyla JF, Bole GG, et al. The relationship of dermatomyositis and polymyositis to internal malignancy. Arch Derm
1980;116:295-8.
20. Lakhanpal S, Bunch TW, Ilstrup DM, et al. Polymyositis-dermatomyositis and malignant lesions: does an association exist?
Mayo Clin Proc 1980;616:45-53.
21. Callen JP. The value of malignancy evaluation in patients with dermatomyositis. J Am Acad Dermatol 1982;6:163-9.
22. Richardson JB, Callen JP. Dermatomyositis and malignancy. Med Clin North Am 1989;73:1211-20.
23. Dalakas MC. Polymyositis, dermatomyositis and inclusion-body myositis. N Engl J Med 1991;323:1487-1508.
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