Objective: To examine the clinical utility of tumor markers in dermatomyositis/polymyositis patients in Taiwan.
Method: Data were collected retrospectively from the database of Taichung Veterans General Hospital in Taiwan from 1998 to 2014. Patients who fulfilled Bohan and Peter criteria of dermatomyositis/polymyositis were recruited. Serum level of tumor markers including carcinoembryonic antigen, alpha-fetoprotein, carbohydrate antigen 125, carbohydrate antigen 19-9 and carbohydrate antigen 15-3 were measured. The occurrence of malignancies and interstitial lung disease was identified. The association of tumor markers with malignancies and interstitial lung disease was examined using Chi-square test or Fisher's exact test.
Results: Among the enrolled 151 patients, 98 (64.9%) dermatomyositis and 53 (35.1%) polymyositis, a total of 15 malignancies were detected: breast ductal carcinoma (n = 4), bladder transitional cell carcinoma (n = 2), lung adenocarcinoma (n = 2), cervical intraepithelial neoplasia 3 and papillary squamous cell carcinoma (n = 2), colorectal (colon and rectal adenocarcinoma) (n = 2), uterine adenocarcinoma (n = 1), nasopharyngeal carcinoma (n = 1) and hematological malignancy (myelodysplastic with excess blast cells) (n = 1). Among the patients with malignancies, 13 (86.7%) had dermatomyositis, 2 (13.3%) polymyositis and 3 (20%) interstitial lung disease. The mean duration from dermatomyositis/polymyositis diagnosis to the occurrence of malignancies was 6.05 ± 5.69 years. There was no significant association of raised tumor markers with the occurrence of malignancies (p > 0.085), while a significant association was observed between the elevated levels of carbohydrate antigen 15-3 and the presence of interstitial lung disease (p = 0.006).
Conclusion: Tumor markers were not useful in malignancy screening or dermatomyositis/polymyositis patients in this tertiary center. The evaluation of the occurrence of malignancy in dermatomyositis/polymyositis patient should include a multidimensional approach. A raised level of carbohydrate antigen 15-3 may be a potential indicator of the presence of interstitial lung disease in dermatomyositis/polymyositis patients.
Adult-onset dermatomyositis has been found to be associated with underlying malignancies in up to 40% of patients. The aims of this study were to determine the demographic profile, the underlying cancer and outcome in patients with dermatomyositis. This was a retrospective review of 38 patients with dermatomyositis seen over a 12-year period in the Department of Dermatology, Kuala Lumpur Hospital. Of these, 18 (47.4%) had an associated underlying malignancy. The male to female ratio was 2:1. Ten patients (55.6%) were Chinese. The youngest patient encountered was 32 years old. Nasopharyngeal carcinoma (61.1%) was the most common malignancy in our study population. Tumour markers were not useful as the initial screening for malignancies. Thorough screening for malignancy is imperative in adult dermatomyositis especially those above 40 years old.
AIM: The objective of our review is to investigate the association between dermatomyositis patients and nasopharyngeal carcinoma (NPC) together with the clinical presentation of the patients and their management in otorhinolaryngology.
BACKGROUND: NPC is a malignant disease with good prognosis on early diagnosis. However, the relationship between the dermatomyositis and NPC and its management is not well defined.
MATERIALS AND METHODS: A 10-year retrospective review of case records of 21 dermatomyositis patients seen in Otorhinolaryngology Department of Hospital Selayang from January 2000 to November 2010.
RESULTS: These patients ranged from 19 to 74 years old and a total of 8 (38%) out of 21 adults with dermatomyositis were detected to have malignancy. Five out of 8 patients had NPC (62.5%). The mean age of patients with NPC and dermatomyositis was 48 years. NPC is diagnosed in 4 out of 5 patients (80%) in the first year of diagnosis of dermatomyositis. The clinical findings of the examination of nasopharynx ranged from hyperemia to exophytic nasopharyngeal mass. Histologically, it is only related to NPC of WHO types II and III.
CONCLUSIONS: There is a strong relationship between dermatomyositis and malignancy, especially NPC. Clinicians should have a high index of suspicion for malignancy in all dermatomyositis patients. Rigid nasoendoscopies and biopsies, serum Epstein-Barr viral capsid IgA antibody and imaging studies are helpful in detecting NPC in dermatomyositis patients.
KEYWORDS: Dermatomyositis; Epstein–Barr viral capsid IgA; Nasopharyngeal carcinoma; Paraneoplastic; WHO histology
'Amyotrophic dermatomyositis' (ADM) is used to describe a small subgroup of patients with the typical skin rash associated with dermatomyositis but without muscle involvement. Lung involvement in ADM is rare. We report on the management of a patient with ADM associated with pulmonary fibrosis at presentation, and her response to corticosteroid treatment.
Dermatomyositis is often presented as paraneoplastic syndrome. The diagnosis of dermatomyositis can prompt clinicians to further investigate the underlying cause, in particular malignancy. This case report illustrates the association of lung adenocarcinoma and dermatomyositis with antecedent presentation of cutaneous and musculoskeletal manifestations, one year prior to the diagnosis of carcinoma.
Paraneoplastic neurological disorder (PND) is a condition due to immune cross-reactivity between the tumour cells and the normal tissue, whereby the "onconeural" antibodies attack the normal host nervous system. It can present within weeks to months before or after the diagnosis of malignancies. Nasopharyngeal carcinoma is associated with paraneoplastic syndrome, for example, dermatomyositis, and rarely with a neurological disorder. We report on a case of nasopharyngeal carcinoma with probable PND. Otolaryngologists, oncologists and neurologists need to be aware of this condition in order to make an accurate diagnosis and to provide prompt treatment.
Eight cases of dermatomyositis were seen in the Skin Department, Hospital Besar Kuala Lumpur between 1989 and 1993. There was one case of juvenile dermatomyositis. There was a majority of Chinese patients (87.5%). There were three patients (37.5%) with underlying malignancies, two of which (66.7%) were nasopharyngeal carcinomas; all were under the age of fifty years. It is important to screen all adult patients for underlying malignancies, even those in the younger age group.
Dermatomyositis is a rare rheumatic disease which predominantly affects the muscles and skin requiring a protracted course of immunosuppressants which may predispose the patients to opportunistic infections. A 49-year-old lady was diagnosed to have dermatomyositis in August 2010 based on history, significantly raised creatine kinase level and muscle biopsy findings. She had recurrent admissions due to fever, myalgia and muscle weakness. She had spiking temperature despite high dose steroids, broad-spectrum antibiotics and antifungal agents. This prompted extensive investigation which leads us to the additional diagnosis of disseminated tuberculosis involving the lungs, muscles and bones. This case demonstrates the challenge in controlling the disease activity of dermatomyositis with immunosuppressants in the setting of disseminated tuberculosis.
We performed a cross-sectional study of the demography, clinical and laboratory features of patients with polymyositis and dermatomyositis followed up in our centre from 2006 to 2009. There were 12 cases, with the majority of them (58.3%) being woman. They have a mean age of 57.8 years and mean disease duration of 11.83 (SD 9.92) months. Our patients comprised of multi-ethnic groups with predominantly Chinese (83.3%), Sarawak natives (8.3%) and Malays (8.3%). They have a mean lag time to diagnosis of 3.67 (SD 4.27) months. Nine (75%) patients had dermatomyositis and 3(25%) had polymyositis. The common clinical manifestations found in our patients were proximal myopathy (100%), neck weakness (33.3%), dysphagia (33.3%) and interstitial lung disease (33.3%). For the nine patients with dermatomyositis, the most common dermatological manifestations were shawl sign (88.9%) and V sign (88.9%). Muscle enzymes were raised in 91.7% of patients. Electromyographies were carried out in four patients, and only one of our patients had muscle biopsy. Only 41.7% of our patients have positive ANA. The majority received prednisolone (100%) and hydroxychloroquine (58.3%). Malignancy occurred in five (three nasopharyngeal carcinomas, one sigmoid colon cancer and one lung cancer) out of the nine dermatomyositis patients but none in the polymyositis group. The mortality rate in our group was 4(33.3%) over the 4-year period. This study demonstrated the rarity of PM/DM in our centre with considerable lag time to diagnosis in our patients. Despite lack of muscle biopsy in our centre, our centre achieved appropriate diagnosis and management of PM/DM.
Juvenile dermatomyositis (JDM) is a systemic autoimmune condition with myopathy. Gastrointestinal and pulmonary manifestations are rare presentation of JDM. Gastrointestinal perforation incidence in JDM is associated with vasculopathy and ischaemia. There are only few reported case of management of JDM with gastrointestinal complication. Management of such condition is challenging. We present a 21-year-old man with spontaneous descending colon perforation undergoing Hartmann's procedure. He subsequently presented with recurrent retroperitoneal abscess at five and 30 months following the initial presentation which was treated with percutaneous drainage. A high index of suspicion is necessary in JDM patients presenting with acute abdomen.
Myopathies, although presenting more commonly in the younger age group, can occur and contribute significantly to disability in the elderly. To describe the spectrum of elderly myopathies, we reviewed 52 elderly patients (> 65 years) from the University of Malaya Medical Centre muscle biopsy databank, constituting 6.8% of 759 adult patients (> 18 years) who underwent muscle biopsy between 1992 and 2012. Commonest were the inflammatory myopathies (41/52, 78.8%), of which 43.9% had dermatomyositis; 23.9% polymyositis; 14.6% sporadic inclusion body myositis; 9.8% undifferentiated myositis and 2.4% overlap myositis. Seven patients (13.4%) had genetic myopathy; 2 muscular dystrophy and 5 chronic progressive external ophthalmoplegia, while 4 patients (7.7%) had drug-associated myopathy, 3 with statins. Malignancies were seen in 9.8% of inflammatory myopathies at diagnosis. Both acquired and genetic myopathies are seen in elderly Malaysians of all ethnicities and should not be misdiagnosed as some are potentially treatable and/or associated with malignancy.
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by progressive muscle weakness and pathognomonic skin eruptions. Systemic corticosteroid with or without an immunosuppressive agent is the current treatment of choice in most cases. Cutaneous disease in DM is often refractory and can become the most challenging component to manage effectively. Here, we report a case of recalcitrant DM in a 66-year-old female who sought chiropractic attention for recent episodes of pain and paresthesia in the neck and exacerbation of joint pain. As expected, the musculoskeletal complaints including neck pain, peripheral arthralgia, and muscle weakness that resolved within 1 month after starting treatment. Unexpectedly, dramatic remission of the characteristic skin rashes occurred concurrently. The underlying therapeutic mechanisms of chiropractic remain elusive. This case highlights the importance of family physicians becoming familiar with diagnosing the condition and using a multidisciplinary team approach to treat recalcitrant DM.
Patients with breast cancer normally present with breast lump or abnormal mammogram. Dermatomyositis is rarely the first presentation. We present a case of a 63-year-old woman who had generalised dermatitis, progressive fatigue and muscle weakness. She was first diagnosed as dermatomyositis and subsequently breast cancer. Her rash and muscle weakness progressed drastically over a month. Tumescent mastectomy and axillary surgery was performed, which led to gradual regression of her dermatomyositis over six months. This case report emphasized in the benefit of early diagnosis and treatment of dermatomyositis and breast cancer. Pros and cons of tumescent mastectomy is discussed as well.
Objectives: To examine the descriptive epidemiology of the patient population referred to paediatric rheumatology centres (PRCs) in Southeast Asia (SEA) and to compare the frequency of conditions encountered with other PRC populations.
Methods: A web-based Registry for Childhood Onset Paediatric Rheumatic Diseases was established in 2009 and seven PRCs in four SEA countries, where paediatric rheumatologists are available, participated in a prospective 24 month data collection (43 months for Singapore).
Results: The number of patients analysed was 4038 (788 from Malaysia, 711 from the Philippines, 1943 from Singapore and 596 from Thailand). Over 70% of patients evaluated in PRCs in Malaysia, the Philippines and Thailand had rheumatic diseases (RDs), as compared with one-half of the proportion seen in Singaporean PRCs, which was similar to the Western PRC experience. Among RDs diagnosed (n = 2602), JIA was the most common disease encountered in Malaysia (41%) and Thailand (61%) as compared with systemic vasculitides in the Philippines (37%) and Singapore (35%) among which Henoch-Schönlein purpura was the most prevalent. SLE and related diseases were more common, but idiopathic pain syndrome and abnormal immunological laboratory tests were rarer than those seen in the West. JIA subtype distributions were different among countries. Among non-RDs (n = 1436), orthopaedic and related conditions predominated (21.7-59.4%).
Conclusion: The frequencies of RDs seen by SEA PRCs were different from those in the West. Systemic vasculitides and SLE were common in addition to JIA. Paediatric rheumatologist availability and healthcare accessibility partially explain these observed discrepancies.
Study site: multination + Selayang Hospital, Malaysia
Introduction: Presentation of Dermatomyositis with Interstitial Lung Disease (ILD) is not uncommon. Case descrip-tion: A 50 years old lady with dyspnoea of 1 month. On examination patient had heliotrope rash around face with gottron papules and fine crepitations bibasally over lung auscultation with proximal weakness of the limbs. High Resolution Computer Tomography of Chest(HRCT of Chest) revealed ground glass appearance with fibrotic changes in both lungs. Blood test serum ANA 1:160, Anti MDA5 strong positive, Anti Ro 52 positive with Serum dsDNA nega-tive. Skin Biopsy-compatible with cutaneous manifestation of dermatomyositis. EMG revealed there is electrophysio-logical cause of myopathy. Lung function test was unable to perform due to severe pain. Investigate for the possibility of underlying malignancy were taken with Serum Tumour Markers, Nasoscopy, ultrasound of pelvis, OGDS and colonoscopy. Did not reveal lesions suspicious of malignancy. Patient treated by using tablet prednisolone 50mg once daily with tapering done and tablet azathioprine 100mg once daily. Planned for IV Methylprednisolone if wors-ening of disease. Patient passed away due to rapidly worsening ILD. Conclusion: Amyotrophic Dermatomyositis with interstitial lung disease(ILD) diagnosis can be confidently diagnosed with clinical correlation and Serum AntiMDA5.
For more than 50 years, nasopharyngeal carcinoma (NPC) has been associated with dermatomyositis (DM), a rare idiopathic inflammatory disorder that mainly affects the skin and muscles. Although the association between these rare diseases is well-documented, the actual prevalence of NPC in DM patients remains unknown. Here, a systematic review and meta-analysis of published data was conducted in accordance with the guidelines of Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA). Electronic databases including PubMed, Scopus, ScienceDirect, and Google Scholar were searched without year or language restrictions for studies reporting the occurrence of NPC in DM patients. The study protocol was lodged with PROSPERO (CRD42021225335). A total of 95 studies covering 303 cases of NPC among 16,010 DM patients was included. Summary estimates were calculated using the random-effects model. The pooled prevalence of NPC in DM was 3.3% (95% CI, 2.5-4.3). When stratified according to study location, higher prevalence estimates were obtained for Hong Kong (36.5%), Malaysia (27.7%), and Singapore (11.9%). There was a predominance of cases among male DM patients compared with females, and most patients were aged 40 and above. Many of the NPC cases were found to be diagnosed after the diagnosis of DM. It is therefore pertinent to screen for NPC in DM patients, especially among older DM patients in the Asian region.
Cytomegalovirus (CMV) retinitis occurs predominantly in Human Immunodeficiency Virus (HIV) -infected patients. It was also reported in HIV-seronegative patients with systemic autoimmune disorder requiring systemic immunosuppression, organ or bone marrow transplantation, haematological or breast malignancy receiving chemotherapy, ocular diseases following intraocular or periocular corticosteroid injection, diabetes mellitus and Good syndrome. However, CMV retinitis in patients with concurrent dermatomyositis and malignancy has not been previously reported. It has not been reported in cancer other than haematological or breast malignancy, or in cancer patient prior to chemotherapy. We report a case of 40-year-old HIV-seronegative woman with underlying dermatomyositis and lung malignancy who developed right CMV retinitis which relapsed after recommencement of immunosuppressant. Both episodes of CMV retinitis were successfully treated after taken her immunocompromised state into consideration.