A Case of Anti-SAE1 Dermatomyositis

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
Physical examination	Heliotrope rash, Gottron’s papules on the dorsal metacarpophalangeal and proximal interphalangeal joint, and an erythematous rash on back and upper leg. There was no muscle weakness
Laboratory test results	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 9 µmol/L; CK 198 IU/L; complement analysis: normal; 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
Immunological laboratory tests	Systemic sclerosis antibodies: negative; Positive myositis antibodies: anti-SAE1; 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
Histology	Skin biopsy: H&E staining showed vacuolar changes in the basal layer of the epidermis and perivascular 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
Pulmonary function testing	2018: vital capacity: 2.08 L (70% of the predicted value); diffusing capacity for carbon monoxide: 54% of the predicted value
Imaging	See Figure 1
Treatment	Methylprednisolone (1000 mg a day for three days) followed by prednisolone 60 mg every day, which 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, Lübeck, Germany, according to manufacturer’s instructions).