Development of microscopic polyangiitis following idiopathic pleuroparenchymal l fibroelastosis: A case report.

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare type of idiopathic interstitial pneumonia, which is characterised by pleural fibrosis and subjacent parenchymal fibroelastosis of the upper lobes. Herein, we present a case of microscopic polyangiitis (MPA) following PPFE. The patient had abnormal shadows on chest radiographs 15 years before the onset of MPA, and the patient was diagnosed with PPFE. Four years after the PPFE diagnosis, the patient was diagnosed with MPA based on persistent fever, purpura, mononeuritis multiplex, myeloperoxidase-antineutrophil cytoplasmic antibody positivity, and pathological findings of peritubular capillaritis on kidney biopsy. The patient was treated with glucocorticoids, including methylprednisolone pulse therapy and rituximab, followed by maintenance therapy with rituximab. One year after treatment, the PPFE had not worsened. PPFE occasionally occurs secondary to connective tissue disease, including MPA; however, to the best of our knowledge, this is the first report of PPFE preceding MPA. Our case suggests that PPFE, as other interstitial lung diseases, may be associated with MPA and precede the onset of MPA. The accumulation of more cases is needed to clarify the characteristics of MPA-associated PPFE.

A case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura.

An 18-year-old Japanese woman with systemic lupus erythematosus experienced dyspnoea, headache, tinnitus, and purpura for 2 weeks and was admitted to our hospital. The patient had been diagnosed with systemic lupus erythematosus and secondary immune thrombocytopenia 8 years before and treated with high-dose prednisolone and mycophenolate mofetil. Since the blood test on admission showed haemolytic anaemia with a positive direct Coombs test and anti-glycoprotein IIb/IIIa antibodies, the patient was initially diagnosed with Evans syndrome (ES). The patient was treated with pulse intravenous methylprednisolone followed by 45 mg/day prednisolone; however, the patient's platelet count did not normalise. Based on a low level of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) activity and a high level of ADAMTS-13 inhibitors, a diagnosis of acquired thrombotic thrombocytopenic purpura (TTP) was confirmed. After undergoing therapeutic plasma exchange for 6 consecutive days, the patient's platelet count recovered rapidly. Although concurrent acquired TTP and ES have not been reported previously, the findings from this case highlight the importance of measuring ADAMTS-13 activity and inhibitors to rule out acquired TTP, especially when ES is refractory to glucocorticoids.

A case of Takayasu arteritis complicated with acute pericarditis at initial presentation.

Takayasu arteritis (TAK) is a rare, large-vessel vasculitis, frequently presenting at approximately 20 years of age. Patients with TAK without characteristic clinical findings are sometimes left undiagnosed and are followed by a fever of unknown origin; delayed diagnosis may lead to irreversible ischaemia and organ damage. Here, we report a case of an 18-year-old woman with TAK complicated by acute pericarditis at initial presentation. She was diagnosed with idiopathic acute pericarditis and treated with non-steroidal anti-inflammatory drugs (NSAIDs). However, the patient's fever and pain in the chest and upper back persisted. On admission to our hospital, magnetic resonance angiography and ultrasonography revealed wall thickening in the common carotid artery, subclavian artery, and aorta, along with vascular narrowing in the celiac, superior mesenteric, and bilateral renal arteries. The patient was diagnosed with TAK and treated with glucocorticoids, including methylprednisolone pulse therapy, and azathioprine. The treatment improved the patient's signs and symptoms, and pericardial effusion decreased. Acute pericarditis is a rare manifestation of TAK, but it is important to differentiate diseases, including TAK in patients with acute pericarditis who fail to respond to 2-3 weeks of conventional therapy with NSAIDs.

Tumour necrosis factor inhibitor-induced myositis in a patient with ulcerative colitis.

Tumour necrosis factor (TNF) inhibitors are known to induce autoimmune diseases, such as lupus-like syndrome; in rare cases, TNF inhibitor-induced myositis has been reported. This report documents the case of a male patient with ulcerative colitis (UC) complicated by TNF inhibitor-induced myositis. After UC diagnosis and treatment with azathioprine and infliximab, he was evaluated for a recent 5-month history of muscle weakness and pain. Laboratory tests revealed elevated muscle enzymes, such as serum creatine kinase (CK) and aldolase. He also tested positive for anti-nuclear antibodies and anti-double stranded DNA antibodies. High-intensity signals in his quadriceps on magnetic resonance image (MRI) and fibrillation potentials in his proximal muscles on electromyography were demonstrated. Muscle biopsy revealed the endomysial infiltration of mononuclear cells surrounding myofibers. Eventually, the patient fulfilled the classification criteria for idiopathic inflammatory myopathies. Although an adverse drug reaction of infliximab had been speculated, his muscle involvements did not improve in 6 weeks from the last administration of infliximab; therefore, treatment with prednisolone was initiated. Subsequently, his muscle symptoms ameliorated, and his serum CK levels returned to normal. Repeat MRI revealed a complete resolution of the signal intensity, and he reported no symptoms of UC or myositis while prednisolone was tapered without resumption of infliximab. Clinicians should consider the diagnosis of drug-induced myositis if muscle symptoms develop in patients treated with TNF inhibitors.

Serum levels of soluble programmed cell death protein 1 and soluble programmed cell death protein ligand 2 are increased in systemic lupus erythematosus and associated with the disease activity.

OBJECTIVE: Programmed cell death protein 1 (PD-1) pathway plays important roles in systemic lupus erythematosus (SLE). We aimed to elucidate the association of serum soluble PD-1 (sPD-1) and related molecules with SLE and to explore their usefulness as biomarkers. METHODS: We retrospectively measured the serum levels of sPD-1, soluble PD-ligand 1 (sPD-L1), soluble PD-ligand 2 (sPD-L2) and interleukin (IL)-21 by ELISA in SLE patients, systemic sclerosis patients and healthy controls. Repeat sera samples were also obtained post treatment. RESULTS: The serum levels of sPD-1 and sPD-L2 in SLE patients with high disease activity were significantly higher than those in SLE patients with low disease activity, systemic sclerosis patients and healthy controls (n = 58, 15, 20 and 21, respectively; p < 0.001). However, the serum levels of sPD-L1 and IL-21 were not elevated in SLE patients. The serum levels of sPD-1 and sPD-L2 were higher among active SLE patients who tested positive for anti-dsDNA antibodies than in those who tested negative (p = 0.002 and <0.001, respectively). There were moderate correlations between the serum levels of sPD-1 and sPD-L2 and the SLE Disease Activity Index 2000 scores, the titres of anti-dsDNA antibodies and the serum levels of complements. Furthermore, the serum levels of sPD-1 and sPD-L2 decreased significantly in accordance with disease amelioration following treatment (p < 0.001). CONCLUSION: The present study demonstrated the association of serum sPD-1 and sPD-L2 with SLE and suggests their usefulness as disease activity biomarkers for SLE.

Association of Serum Soluble CD163 with Polymyositis and Dermatomyositis, Especially in Anti-MDA5 Antibody-positive Cases.

OBJECTIVE: We elucidated the association of serum soluble CD163 (sCD163) with rapidly progressive interstitial lung disease (RP-ILD), autoantibody profiles, and serum ferritin in patients with polymyositis (PM), classic dermatomyositis (DM), and clinical amyopathic dermatomyositis (CADM). METHODS: Serum sCD163 levels were retrospectively measured by ELISA in patients with PM, classic DM, and CADM, as well as in healthy controls (HC). Repeat sera samples were obtained posttreatment from available patients. The associations between serum sCD163 levels and clinical information were analyzed. RESULTS: Serum sCD163 levels in patients with PM/classic DM/CADM were significantly higher than those in HC (n = 72, 56, 34, and 68, respectively; p < 0.001 for all comparisons). No significant difference was observed between serum sCD163 levels in patients with and without ILD (p = 0.16) or between those with RP-ILD and chronic ILD (p = 0.21). Serum sCD163 levels were significantly higher in patients with anti-MDA5 antibodies (n = 27) than in those without (p = 0.001). Serum sCD163 levels were weakly correlated with serum ferritin levels in the patients with PM, classic DM, and CADM (r = 0.21). Serum sCD163 levels decreased significantly following treatment in all patient groups (p = 0.003). CONCLUSION: The present results suggest an association of serum sCD163 with PM, classic DM, and CADM, especially in anti-MDA5 antibody-positive cases. However, serum sCD163 levels were not specifically associated with ILD or RP-ILD.

Regional overdistension during prone positioning in a patient with acute respiratory failure who was ventilated with a low tidal volume: a case report.

BACKGROUND: Prone positioning may provide a uniform distribution of transpulmonary pressure and contribute to prevent ventilator-induced lung injury. However, despite moderate positive end-expiratory pressure and low tidal volumes, there is still a risk of regional overdistension. CASE PRESENTATION: A man with refractory hypoxemia was mechanically ventilated with prone positioning. Although prone positioning with a plateau pressure of 18 cmH2O and a positive end-expiratory pressure of 8 cmH2O promptly improved oxygenation, regional ventilation monitoring using electrical impedance tomography initially detected decreased distribution in the dorsal region but increased in the ventral, suggesting overdistension. CONCLUSIONS: Our experience indicates monitoring regional ventilation distribution is useful for decreasing the risk of overdistension during prone positioning.

[A case of propranolol therapy for infantile capillary hemangiomas of the parotis].

A 4-month-old healthy female infant presented with rapid onset of subaural swelling over a three-month period. A head and neck exam demonstrated a subaural elastic hard mass with a red birthmark below the left auricle. MRI of the neck demonstrated a well-defined parotid mass consistent with a haemangioma. We treated this infant with 1 mg/kg of propranolol, which was gradually increased over two months to a dose of 2 mg/kg daily. The tumor began to reduce in size within three days after drug administration, and became less prominent in one month, and had almost totally disappeared within four months. On ten-month follow-up, the patient was asymptomatic and repeated MRI demonstrated further regression of the tumor. Propranolol could be the first-line choice for treating haemangioma rather than simple