A man in his 40s with a history of congenitally corrected transposition of the great arteries (CCTGA) and closure of ventricular septal defect was referred to our hospital with purpura and hematuria. Presence of purpura, renal damage, and pathological findings on skin biopsy led to the diagnosis of IgA vasculitis (IgAV). Oral prednisolone (PSL) was initiated. However, Streptococcus pseudoporcinus was isolated from blood cultures, and transthoracic echocardiogram revealed vegetation on the pulmonary valve. From these findings, the diagnosis of infective endocarditis (IE) was made. Although the patient's condition improved after PSL interruption and antibiotic administration, his purpura relapsed. PSL readministration improved symptoms, with no further relapse even after gradual PSL dose reduction. The present case raises awareness of the importance of recognizing the occurrence of IE in IgAV patients, especially in those with congenital heart disease. CCTGA should be acknowledged as a risk factor for IE in the right-sided heart.

No abstract

A 49-year-old Japanese man with a 2-month history of a fever, headache, and bilateral conjunctival hyperemia was admitted. His condition fulfilled the giant cell arteritis classification criteria (new headache, temporal artery tenderness, elevated ESR) and atypical Cogan's syndrome (CS) with scleritis and sensorineural hearing loss (SNHL). The interleukin (IL)-6 serum level was extremely high. Two weeks after his insufficient response of SNHL and scleritis to oral prednisolone, we administered tocilizumab (TCZ); rapid improvements in scleritis and SNHL occurred. Early IL-6 target therapy can help prevent irreversible CS-induced sensory organ damage.

A 41-year-old woman with systemic lupus erythematosus (SLE) was admitted to our hospital due to a fever at 35 weeks of pregnancy. Laboratory testing revealed a low platelet count and elevated liver enzymes. Emergency Caesarean section was performed due to the risk of SLE exacerbation or hemolytic anemia, elevated liver enzyme, and low platelet count syndrome. Based on the blood culture results, the patient was diagnosed with Listeria monocytogenes bacteremia. She was treated with ampicillin and eventually recovered, and the neonate did not have any complications. Pregnant women with SLE are at risk of complications. Listeriosis should be monitored for and, if found, managed appropriately.

A 54-year-old woman developed drop head syndrome (DHS), Raynaud's phenomenon and creatine kinase (CK) elevation. She did not meet the international classification criteria of dermatomyositis/polymyositis, as we observed no muscle weakness, grasping pain or electromyography abnormality in her limbs, and antiaminoacyl tRNA synthetase (ARS) antibody was negative. Cervical magnetic resonance imaging and a muscle biopsy of the trapezius muscle revealed myositis findings as the only clinical observations in muscle. These findings, along with her anti-U1-ribonucleoprotein (RNP) antibody positivity and leukopenia, resulted in a diagnosis of mixed connective tissue disease (MCTD). Prednisolone treatment significantly improved her myositis. To our knowledge, this is the first report of DHS as the only muscle complication of MCTD.