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A Case of Anti-SAE1 Dermatomyositis

A Case of Anti-SAE1 Dermatomyositis Hindawi Case Reports in Immunology Volume 2022, Article ID 9000608, 4 pages https://doi.org/10.1155/2022/9000608 Case Report 1 2 3 4 Max de Vries , Marco W. J. Schreurs , Els J. M. Ahsmann, Marcela Spee-Dropkova, and Faiz Karim Department of Internal Medicine, Groene Hart Hospital, Bleulandweg 10 2803 HH, Gouda, Netherlands Department of Immunology, Erasmus MC, University Medical Center, Postbus 2040 3000 CA, Rotterdam, Netherlands Department of Clinical Pathology, Groene Hart Hospital, Bleulandweg 10 2803 HH, Gouda, Netherlands Department of Radiology, Groene Hart Hospital, Bleulandweg 10 2803 HH, Gouda, Netherlands Correspondence should be addressed to Max de Vries; max_de_vries1@hotmail.com Received 17 August 2021; Accepted 15 February 2022; Published 4 March 2022 Academic Editor: Jiri Litzman Copyright © 2022 Max de Vries et al. *is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Introduction. Anti-SAE1 antibodies have a low prevalence in dermatomyositis patients. Case Description. A 61-year-old woman presented with progressive shortness of breath, arthralgia, heliotrope rash, Gottron’s papules, and erythematous rash. She had an interstitial lung disease (ILD) with a significant decrease in lung function. *ere was no muscle involvement. Immunological laboratory test results showed strongly positive anti-SAE1 antibodies. Glucocorticoid treatment resulted in remission of der- matomyositis. Conclusion. Anti-SAE antibodies in dermatomyositis patients are closely linked to unique clinical features. 1. Introduction 2. Case Presentation *e patient characteristics and the main clinical features are Dermatomyositis is an idiopathic inflammatory myopathy with a wide variability in clinical presentation due to a range given in Table 1. A 61-year-old Caucasian woman with a medical history of hypertension, interstitial cystitis, and of cutaneous and systemic manifestations. *is includes anteroseptal myocardial infarction presented to our out- variety in muscle and joint manifestation, pulmonary patient clinic. For her comorbidities, she used the following manifestation, and association with malignancy. *is in- medication: carbasalate calcium, perindopril, nitro- consistency creates a diagnostic challenge for physicians. furantoin, celecoxib, and amitriptyline. She presented with Myositis-specific autoantibodies (MSAs) are antibodies progressive shortness of breath, fatigue, arthralgia, and skin which are only found in inflammatory myopathies, and alterations. *e skin alterations were the initial complaints; some MSAs are specific for dermatomyositis [1]. Every MSA she suffered from and was thought to have antibiotic hy- is closely linked to both unique clinical features, response to persensitivity. She was treated with amoxicillin clavulanate treatment and prognosis [2]. In 2007, antismall ubiquitin- for pneumonia. However, skin alterations persisted after like modifier 1-activating enzyme (SAE1) antibodies were identified as a dermatomyositis-specific MSA [3]. Its fre- discontinuation of the antibiotics. She had no complaints of dysphagia, muscle pain, or proximal muscle weakness. Her quency among dermatomyositis patients is very low, ranging vital signs were normal. Physical examination revealed a from 1.5% to 8.0% [4]. As few patients with anti-SAE1 heliotrope rash and Gottron’s papules on the dorsal meta- dermatomyositis have been described, it is important to carpophalangeal and proximal interphalangeal joints and an describe more cases of this rare subtype. We describe one erythematous rash on her back and upper leg. Laboratory patient with anti-SAE1 dermatomyositis. 2 Case Reports in Immunology Table 1: Characteristics and the main clinical features of the patient. Gender Female Age 61 years Medical history Hypertension, interstitial cystitis, and anteroseptal myocardial infarction Symptoms Progressive shortness of breath, fatigue, arthralgia, and skin alterations Heliotrope rash, Gottron’s papules on the dorsal metacarpophalangeal and proximal interphalangeal joint, Physical examination and an erythematous rash on back and upper leg. *ere was no muscle weakness 2018: hemoglobin 8.8 mmol/L; leucocytes 4.4 10∗ 9/L; CRP 5 mg/L; ASAT 332 IU/L; ALAT 282 IU/L; LDH 377 IU/L; ALP 170 IU/L; GGT 101 IU/L; bilirubin 9µmol/L; CK 198 IU/L; complement analysis: normal; Laboratory test results ANA: positive (nuclear dense speckled (AC-2) and nuclear fine speckled (AC-4) pattern); ANCA (MPO/ PR3): normal; rheumatoid factor IgM: normal; CCP antibody: normal Systemic sclerosis antibodies : negative Immunological laboratory Positive myositis antibodies : anti-SAE1 tests Negative myositis antibodies : anti-OJ; anti-EJ; anti-PL-12; anti-PL-7; anti-SRP; anti-Jo-1; anti-PM-Scl75; anti-PM-Scl100; anti-Ku; anti-NXP2; anti-MDA5; anti-TIF-1 gamma; anti-Mi-2 beta; anti-Mi-2 alpha Skin biopsy: H&E staining showed vacuolar changes in the basal layer of the epidermis and perivascular Histology lymphocytic infiltration of the dermis. Immunofluorescence: dispositions of IgA, IgG, and IgM in the basal membrane which is compatible with dermatomyositis and cutaneous lupus erythematosus 2018: vital capacity: 2.08 L (70% of the predicted value); diffusing capacity for carbon monoxide: 54% of the Pulmonary function testing predicted value Imaging See Figure 1 Methylprednisolone (1000 mg a day for three days) followed by prednisolone 60 mg every day, which Treatment eventually was tapered down to 5 mg every day. Azathioprine was discontinued because of thrombocytopenia CRP, C-reactive protein; ASAT, aspartate aminotransferase; ALAT, alanine aminotransferase; LDH, lactate dehydrogenase; ALP, alkaline phosphatase; GGT, gamma-glutamyl transpeptidase; CK, creatinine kinase; ANA, antinuclear antibodies; ANCA, antineutrophil cytoplasmic antibodies; CCP, cyclic citrullinated peptide. Systemic sclerosis and myositis antibodies were determined by line immune assay (Euroline profile, Euroimmun, Lubeck, ¨ Germany, according to manufacturer’s instructions). test results are given in Table 1. Pulmonary function testing dermatomyositis is in remission. Last control was in No- showed a vital capacity of 2.08 L, which was 70% of the vember 2021 (2 years after start of treatment). predicted value, and a diffusing capacity for carbon mon- oxide of 54% of the predicted value. CT scan showed diffuse 3. Discussion consolidations, suspect for interstitial lung disease (ILD) (Figure 1(a)). Anti-SAE1 antibodies are relatively rare in dermatomyositis Skin biopsy hematoxylin and eosin (H&E) staining patients with a very low prevalence ranging between 1.5 and showed vacuolar changes in the basal layer of the epidermis 8.0%, and only few patients have been described so far. *ere and mild perivascular lymphocytic infiltration of the dermis is heterogeneity in clinical features among patients. More- (Figure 2(a)). Direct immunofluorescence showed depositions over, there seems to be difference in clinical presentation of IgA, IgG, and IgM in the basal membrane zone which is between Western and Asian populations, which creates a compatible with dermatomyositis and cutaneous lupus diagnostic challenge for clinicians [5]. erythematosus. Immunological laboratory test results showed Cutaneous disease was the initial manifestation of the strongly positive anti-SAE1 antibodies. *e antinuclear an- dermatomyositis in our patient, which is a frequent tibodies (ANA) determination by indirect immunofluores- finding in anti-SAE1-positive dermatomyositis patients. cence (IIF) on HEp-2 cells is shown in Figure 2(b). *is shows However, there is also a subset which presents with skin a positive ANA with both nuclear dense speckled (AC-2) and and muscle disease simultaneously. Typical findings in nuclear fine speckled (AC-4) pattern, the latter being com- anti-SAE1 positive patients are heliotrope rash, Gottron’s patible with anti-SAE1 reactivity. sign, and papules. An erythematous rash is also described Our patient was admitted to the hospital for methyl- in a subset of patients [1]. Dysphagia is present in 47% of prednisolone treatment (1000 mg a day for three days). An patients [5]. abdominal ultrasound was preformed to screen for malig- *e prevalence of ILD among anti-SAE1 positive pa- nancy and because of the elevated liver enzymes, but no tients is nearly 50% and is usually mild, with few patients abnormalities were found. After three days of treatment with having respiratory complaints despite having abnormalities methylprednisolone, we started prednisolone 60 mg every on imaging, which are compatible with ILD. However, our day, which eventually was tapered down to 5 mg every day. patient did experience respiratory symptoms and had sig- She was shortly treated with azathioprine. *is was dis- nificant decrease in lung function. *e characteristics of ILD continued due to thrombocytopenia. Monotreatment with in anti-SAE1 patients on chest HRCT are subpleural pe- low-dose prednisone 5 mg/day was, however, sufficient. *e ripheral-dominant small ground glass opacities, corre- elevated liver enzymes abated during treatment. *e sponding to organizing pneumonia [2]. Case Reports in Immunology 3 (a) (b) Figure 1: Imaging studies of the patient. (a) CT-thorax 2018 (pretreatment): bilateral patchy consolidations with distortion and dilatation of the bronchioles and parenchymal distortion with peripheral subpleural and peribronchovascular distribution, which are compatible with an organizing pneumonia. (b) CT-thorax 2019 (posttreatment): bilateral atelectasis with traction bronchiectasis, which are compatible with residual abnormalities after an organizing pneumonia. (a) (b) Figure 2: Skin biopsy and ANA determination. (a) H&E staining showing vacuolar changes in the basal layer of the epidermis (arrow) and perivascular lymphocytic infiltration of the dermis. (b) *e IIF HEp-2 pattern showing a positive ANA with combined nuclear dense (AC-2) and fine speckled (AC-4) pattern (http://www.ANApatterns.org), the latter being compatible with anti-SAE1 reactivity. Arrows indicate fine speckled nucleoplasm in mitotic cells outside chromatin mass. As other MSAs, anti-SAE1 antibodies may be associated immunoglobulins) are described as treatment for anti-SAE1 with an increased risk of cancer. *erefore, tumor screening positive patients [4]. is recommended after initial diagnosis in all dermatomyo- In conclusion, anti-SAE1 antibodies are relatively rare in dermatomyositis patients. Classical findings in anti-SAE1 sitis patients [6]. Most studies on anti-SAE1 antibodies and positive patients are heliotrope rash, Gottron’s sign, and cancer show a positive association [5, 7–9]. All anti-SAE1 papules, muscle weakness, and dysphagia. Additional findings positive patients who developed cancer suffered from ade- include mild interstitial lung disease, and there may be an nocarcinoma. *ese adenocarcinomas were from cervical, increased risk of cancer. Glucocorticoid treatment is the pulmonary, esophageal, or rectal origin. *e frequency of preferred first-line treatment in dermatomyositis patients. cancer in anti-SAE1-positive patients in studies included by Lu et al. ranged between 14% and 57% [10]. However, a Data Availability common denominator of these studies was a small sample size. *e clinical data used to support the findings of this study Glucocorticoid treatment is the preferred treatment in are included within the article. dermatomyositis patients. Both monotherapy with gluco- corticoids as combined treatment with glucocorticoid and Conflicts of Interest immunosuppressive agents (methotrexate, cyclophospha- mide, cyclosporine, tacrolimus, rituximab, thalidomide, or *e authors declare that they have no conflicts of interest. 4 Case Reports in Immunology References [1] P. W. Wolstencroft and D. F. Fiorentino, “Dermatomyositis clinical and pathological phenotypes associated with myositis- specific autoantibodies,” Current Rheumatology Reports, vol. 20, p. 28, 2018. [2] T. Gono, Y. Tanino, A. Nishikawa et al., “Two cases with autoantibodies to small ubiquitin-like modifier activating enzyme: a potential unique subset of dermatomyositis-asso- ciated interstitial lung disease,” International Journal of Rheumatic Diseases, vol. 22, pp. 1582–1586, 2019. [3] Z. E. Betteridge, H. Gunawardena, H. Chinoy et al., “Clinical and human leucocyte antigen class II haplotype associations of autoantibodies to small ubiquitin-like modifier enzyme, a dermatomyositis-specific autoantigen target, in UK Caucasian adult-onset myositis,” Annals of the Rheumatic Diseases, vol. 68, no. 10, pp. 1621–1625, 2009. [4] E. Jia, J. Wei, H. Geng et al., “Diffuse pruritic erythema as a clinical manifestation in anti-SAE antibody-associated der- matomyositis: a case report and literature review,” Clinical Rheumatology, vol. 38, no. 8, pp. 2189–2193, 2019. [5] Y. Ge, X. Lu, X. Shu, Q. Peng, and G. Wang, “Clinical characteristics of anti-SAE antibodies in Chinese patients with dermatomyositis in comparison with different patient co- horts,” Scientific Reports, vol. 7, pp. 188–8, 2017. [6] N. Schlecht, C. Sunderkotter, ¨ S. Niehaus, and D. Nashan, “Update on dermatomyositis in adults,” JDDG: Journal der Deutschen Dermatologischen Gesellschaft, vol. 18, no. 9, pp. 995–1013, 2020. [7] E. Tarricone, A. Ghirardello, M. Rampudda, N. Bassi, L. Punzi, and A. Doria, “Anti-SAE antibodies in autoimmune myositis: identification by unlabelled protein immunoprecipitation in an Italian patient cohort,” Journal of Immunological Methods, vol. 384, no. 1-2, pp. 128–134, 2012. [8] M. Fujimoto, T. Matsushita, Y. Hamaguchi et al., “Autoan- tibodies to small ubiquitin-like modifier activating enzymes in Japanese patients with dermatomyositis: comparison with a UK Caucasian cohort,” Annals of the Rheumatic Diseases, vol. 72, no. 1, pp. 151–153, 2013. [9] Y. Muro, K. Sugiura, and M. Akiyama, “Low prevalence of anti-small ubiquitin-like modifier activating enzyme anti- bodies in dermatomyositis patients,” Autoimmunity, vol. 46, no. 4, pp. 279–284, 2013. [10] X. Lu, Q. Peng, and G. Wang, “*e role of cancer-associated autoantibodies as biomarkers in paraneoplastic myositis syndrome,” Current Opinion in Rheumatology, vol. 31, no. 6, pp. 643–649, 2019. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Case Reports in Immunology Hindawi Publishing Corporation

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Abstract

Hindawi Case Reports in Immunology Volume 2022, Article ID 9000608, 4 pages https://doi.org/10.1155/2022/9000608 Case Report 1 2 3 4 Max de Vries , Marco W. J. Schreurs , Els J. M. Ahsmann, Marcela Spee-Dropkova, and Faiz Karim Department of Internal Medicine, Groene Hart Hospital, Bleulandweg 10 2803 HH, Gouda, Netherlands Department of Immunology, Erasmus MC, University Medical Center, Postbus 2040 3000 CA, Rotterdam, Netherlands Department of Clinical Pathology, Groene Hart Hospital, Bleulandweg 10 2803 HH, Gouda, Netherlands Department of Radiology, Groene Hart Hospital, Bleulandweg 10 2803 HH, Gouda, Netherlands Correspondence should be addressed to Max de Vries; max_de_vries1@hotmail.com Received 17 August 2021; Accepted 15 February 2022; Published 4 March 2022 Academic Editor: Jiri Litzman Copyright © 2022 Max de Vries et al. *is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Introduction. Anti-SAE1 antibodies have a low prevalence in dermatomyositis patients. Case Description. A 61-year-old woman presented with progressive shortness of breath, arthralgia, heliotrope rash, Gottron’s papules, and erythematous rash. She had an interstitial lung disease (ILD) with a significant decrease in lung function. *ere was no muscle involvement. Immunological laboratory test results showed strongly positive anti-SAE1 antibodies. Glucocorticoid treatment resulted in remission of der- matomyositis. Conclusion. Anti-SAE antibodies in dermatomyositis patients are closely linked to unique clinical features. 1. Introduction 2. Case Presentation *e patient characteristics and the main clinical features are Dermatomyositis is an idiopathic inflammatory myopathy with a wide variability in clinical presentation due to a range given in Table 1. A 61-year-old Caucasian woman with a medical history of hypertension, interstitial cystitis, and of cutaneous and systemic manifestations. *is includes anteroseptal myocardial infarction presented to our out- variety in muscle and joint manifestation, pulmonary patient clinic. For her comorbidities, she used the following manifestation, and association with malignancy. *is in- medication: carbasalate calcium, perindopril, nitro- consistency creates a diagnostic challenge for physicians. furantoin, celecoxib, and amitriptyline. She presented with Myositis-specific autoantibodies (MSAs) are antibodies progressive shortness of breath, fatigue, arthralgia, and skin which are only found in inflammatory myopathies, and alterations. *e skin alterations were the initial complaints; some MSAs are specific for dermatomyositis [1]. Every MSA she suffered from and was thought to have antibiotic hy- is closely linked to both unique clinical features, response to persensitivity. She was treated with amoxicillin clavulanate treatment and prognosis [2]. In 2007, antismall ubiquitin- for pneumonia. However, skin alterations persisted after like modifier 1-activating enzyme (SAE1) antibodies were identified as a dermatomyositis-specific MSA [3]. Its fre- discontinuation of the antibiotics. She had no complaints of dysphagia, muscle pain, or proximal muscle weakness. Her quency among dermatomyositis patients is very low, ranging vital signs were normal. Physical examination revealed a from 1.5% to 8.0% [4]. As few patients with anti-SAE1 heliotrope rash and Gottron’s papules on the dorsal meta- dermatomyositis have been described, it is important to carpophalangeal and proximal interphalangeal joints and an describe more cases of this rare subtype. We describe one erythematous rash on her back and upper leg. Laboratory patient with anti-SAE1 dermatomyositis. 2 Case Reports in Immunology Table 1: Characteristics and the main clinical features of the patient. Gender Female Age 61 years Medical history Hypertension, interstitial cystitis, and anteroseptal myocardial infarction Symptoms Progressive shortness of breath, fatigue, arthralgia, and skin alterations Heliotrope rash, Gottron’s papules on the dorsal metacarpophalangeal and proximal interphalangeal joint, Physical examination and an erythematous rash on back and upper leg. *ere was no muscle weakness 2018: hemoglobin 8.8 mmol/L; leucocytes 4.4 10∗ 9/L; CRP 5 mg/L; ASAT 332 IU/L; ALAT 282 IU/L; LDH 377 IU/L; ALP 170 IU/L; GGT 101 IU/L; bilirubin 9µmol/L; CK 198 IU/L; complement analysis: normal; Laboratory test results ANA: positive (nuclear dense speckled (AC-2) and nuclear fine speckled (AC-4) pattern); ANCA (MPO/ PR3): normal; rheumatoid factor IgM: normal; CCP antibody: normal Systemic sclerosis antibodies : negative Immunological laboratory Positive myositis antibodies : anti-SAE1 tests Negative myositis antibodies : anti-OJ; anti-EJ; anti-PL-12; anti-PL-7; anti-SRP; anti-Jo-1; anti-PM-Scl75; anti-PM-Scl100; anti-Ku; anti-NXP2; anti-MDA5; anti-TIF-1 gamma; anti-Mi-2 beta; anti-Mi-2 alpha Skin biopsy: H&E staining showed vacuolar changes in the basal layer of the epidermis and perivascular Histology lymphocytic infiltration of the dermis. Immunofluorescence: dispositions of IgA, IgG, and IgM in the basal membrane which is compatible with dermatomyositis and cutaneous lupus erythematosus 2018: vital capacity: 2.08 L (70% of the predicted value); diffusing capacity for carbon monoxide: 54% of the Pulmonary function testing predicted value Imaging See Figure 1 Methylprednisolone (1000 mg a day for three days) followed by prednisolone 60 mg every day, which Treatment eventually was tapered down to 5 mg every day. Azathioprine was discontinued because of thrombocytopenia CRP, C-reactive protein; ASAT, aspartate aminotransferase; ALAT, alanine aminotransferase; LDH, lactate dehydrogenase; ALP, alkaline phosphatase; GGT, gamma-glutamyl transpeptidase; CK, creatinine kinase; ANA, antinuclear antibodies; ANCA, antineutrophil cytoplasmic antibodies; CCP, cyclic citrullinated peptide. Systemic sclerosis and myositis antibodies were determined by line immune assay (Euroline profile, Euroimmun, Lubeck, ¨ Germany, according to manufacturer’s instructions). test results are given in Table 1. Pulmonary function testing dermatomyositis is in remission. Last control was in No- showed a vital capacity of 2.08 L, which was 70% of the vember 2021 (2 years after start of treatment). predicted value, and a diffusing capacity for carbon mon- oxide of 54% of the predicted value. CT scan showed diffuse 3. Discussion consolidations, suspect for interstitial lung disease (ILD) (Figure 1(a)). Anti-SAE1 antibodies are relatively rare in dermatomyositis Skin biopsy hematoxylin and eosin (H&E) staining patients with a very low prevalence ranging between 1.5 and showed vacuolar changes in the basal layer of the epidermis 8.0%, and only few patients have been described so far. *ere and mild perivascular lymphocytic infiltration of the dermis is heterogeneity in clinical features among patients. More- (Figure 2(a)). Direct immunofluorescence showed depositions over, there seems to be difference in clinical presentation of IgA, IgG, and IgM in the basal membrane zone which is between Western and Asian populations, which creates a compatible with dermatomyositis and cutaneous lupus diagnostic challenge for clinicians [5]. erythematosus. Immunological laboratory test results showed Cutaneous disease was the initial manifestation of the strongly positive anti-SAE1 antibodies. *e antinuclear an- dermatomyositis in our patient, which is a frequent tibodies (ANA) determination by indirect immunofluores- finding in anti-SAE1-positive dermatomyositis patients. cence (IIF) on HEp-2 cells is shown in Figure 2(b). *is shows However, there is also a subset which presents with skin a positive ANA with both nuclear dense speckled (AC-2) and and muscle disease simultaneously. Typical findings in nuclear fine speckled (AC-4) pattern, the latter being com- anti-SAE1 positive patients are heliotrope rash, Gottron’s patible with anti-SAE1 reactivity. sign, and papules. An erythematous rash is also described Our patient was admitted to the hospital for methyl- in a subset of patients [1]. Dysphagia is present in 47% of prednisolone treatment (1000 mg a day for three days). An patients [5]. abdominal ultrasound was preformed to screen for malig- *e prevalence of ILD among anti-SAE1 positive pa- nancy and because of the elevated liver enzymes, but no tients is nearly 50% and is usually mild, with few patients abnormalities were found. After three days of treatment with having respiratory complaints despite having abnormalities methylprednisolone, we started prednisolone 60 mg every on imaging, which are compatible with ILD. However, our day, which eventually was tapered down to 5 mg every day. patient did experience respiratory symptoms and had sig- She was shortly treated with azathioprine. *is was dis- nificant decrease in lung function. *e characteristics of ILD continued due to thrombocytopenia. Monotreatment with in anti-SAE1 patients on chest HRCT are subpleural pe- low-dose prednisone 5 mg/day was, however, sufficient. *e ripheral-dominant small ground glass opacities, corre- elevated liver enzymes abated during treatment. *e sponding to organizing pneumonia [2]. Case Reports in Immunology 3 (a) (b) Figure 1: Imaging studies of the patient. (a) CT-thorax 2018 (pretreatment): bilateral patchy consolidations with distortion and dilatation of the bronchioles and parenchymal distortion with peripheral subpleural and peribronchovascular distribution, which are compatible with an organizing pneumonia. (b) CT-thorax 2019 (posttreatment): bilateral atelectasis with traction bronchiectasis, which are compatible with residual abnormalities after an organizing pneumonia. (a) (b) Figure 2: Skin biopsy and ANA determination. (a) H&E staining showing vacuolar changes in the basal layer of the epidermis (arrow) and perivascular lymphocytic infiltration of the dermis. (b) *e IIF HEp-2 pattern showing a positive ANA with combined nuclear dense (AC-2) and fine speckled (AC-4) pattern (http://www.ANApatterns.org), the latter being compatible with anti-SAE1 reactivity. Arrows indicate fine speckled nucleoplasm in mitotic cells outside chromatin mass. As other MSAs, anti-SAE1 antibodies may be associated immunoglobulins) are described as treatment for anti-SAE1 with an increased risk of cancer. *erefore, tumor screening positive patients [4]. is recommended after initial diagnosis in all dermatomyo- In conclusion, anti-SAE1 antibodies are relatively rare in dermatomyositis patients. Classical findings in anti-SAE1 sitis patients [6]. Most studies on anti-SAE1 antibodies and positive patients are heliotrope rash, Gottron’s sign, and cancer show a positive association [5, 7–9]. All anti-SAE1 papules, muscle weakness, and dysphagia. Additional findings positive patients who developed cancer suffered from ade- include mild interstitial lung disease, and there may be an nocarcinoma. *ese adenocarcinomas were from cervical, increased risk of cancer. Glucocorticoid treatment is the pulmonary, esophageal, or rectal origin. *e frequency of preferred first-line treatment in dermatomyositis patients. cancer in anti-SAE1-positive patients in studies included by Lu et al. ranged between 14% and 57% [10]. However, a Data Availability common denominator of these studies was a small sample size. *e clinical data used to support the findings of this study Glucocorticoid treatment is the preferred treatment in are included within the article. dermatomyositis patients. Both monotherapy with gluco- corticoids as combined treatment with glucocorticoid and Conflicts of Interest immunosuppressive agents (methotrexate, cyclophospha- mide, cyclosporine, tacrolimus, rituximab, thalidomide, or *e authors declare that they have no conflicts of interest. 4 Case Reports in Immunology References [1] P. W. Wolstencroft and D. F. Fiorentino, “Dermatomyositis clinical and pathological phenotypes associated with myositis- specific autoantibodies,” Current Rheumatology Reports, vol. 20, p. 28, 2018. [2] T. Gono, Y. Tanino, A. Nishikawa et al., “Two cases with autoantibodies to small ubiquitin-like modifier activating enzyme: a potential unique subset of dermatomyositis-asso- ciated interstitial lung disease,” International Journal of Rheumatic Diseases, vol. 22, pp. 1582–1586, 2019. [3] Z. E. Betteridge, H. Gunawardena, H. Chinoy et al., “Clinical and human leucocyte antigen class II haplotype associations of autoantibodies to small ubiquitin-like modifier enzyme, a dermatomyositis-specific autoantigen target, in UK Caucasian adult-onset myositis,” Annals of the Rheumatic Diseases, vol. 68, no. 10, pp. 1621–1625, 2009. [4] E. Jia, J. Wei, H. Geng et al., “Diffuse pruritic erythema as a clinical manifestation in anti-SAE antibody-associated der- matomyositis: a case report and literature review,” Clinical Rheumatology, vol. 38, no. 8, pp. 2189–2193, 2019. [5] Y. Ge, X. Lu, X. Shu, Q. Peng, and G. Wang, “Clinical characteristics of anti-SAE antibodies in Chinese patients with dermatomyositis in comparison with different patient co- horts,” Scientific Reports, vol. 7, pp. 188–8, 2017. [6] N. Schlecht, C. Sunderkotter, ¨ S. Niehaus, and D. Nashan, “Update on dermatomyositis in adults,” JDDG: Journal der Deutschen Dermatologischen Gesellschaft, vol. 18, no. 9, pp. 995–1013, 2020. [7] E. Tarricone, A. Ghirardello, M. Rampudda, N. Bassi, L. Punzi, and A. Doria, “Anti-SAE antibodies in autoimmune myositis: identification by unlabelled protein immunoprecipitation in an Italian patient cohort,” Journal of Immunological Methods, vol. 384, no. 1-2, pp. 128–134, 2012. [8] M. Fujimoto, T. Matsushita, Y. Hamaguchi et al., “Autoan- tibodies to small ubiquitin-like modifier activating enzymes in Japanese patients with dermatomyositis: comparison with a UK Caucasian cohort,” Annals of the Rheumatic Diseases, vol. 72, no. 1, pp. 151–153, 2013. [9] Y. Muro, K. Sugiura, and M. Akiyama, “Low prevalence of anti-small ubiquitin-like modifier activating enzyme anti- bodies in dermatomyositis patients,” Autoimmunity, vol. 46, no. 4, pp. 279–284, 2013. [10] X. Lu, Q. Peng, and G. Wang, “*e role of cancer-associated autoantibodies as biomarkers in paraneoplastic myositis syndrome,” Current Opinion in Rheumatology, vol. 31, no. 6, pp. 643–649, 2019.

Journal

Case Reports in ImmunologyHindawi Publishing Corporation

Published: Mar 4, 2022

References