Impact of systemic steroids combined with immunosuppressive treatment on glaucomatous features in patients with systemic lupus erythematosus

AIM: To evaluate the incidence of increased intraocular pressure (IOP) and glaucomatous changes in systemic lupus erythematosus (SLE) patients in comparison with systemic steroids and immunosuppressive treatment. METHODS: Sixty-two women with SLE were divided into two groups: treated (n=47, 94 eyes) and not treated (n=15, 30 eyes) with systemic glucocorticosteroids (GC; GC-free). Twenty-one individuals in GC group were treated with immunosuppressive agents (immunomodulating and biologic). The visual acuity and IOP with ocular pulsatile amplitude (OPA) measurements, as well as scanning laser polarimetry (GDx) with nerve fiber index (NFI) measurement, spectral domain optical coherence tomography (SD-OCT) of the optic disk with retinal nerve fiber layer (RNFL) analysis and the macular region with ganglion cell analysis (GCA) were performed. RESULTS: Mean IOP values in group with combined GC and immunosuppressive therapy was 15.8±2.56 mm Hg and was significantly lower than in individuals with exclusive GC treatment (17.63±4.38 mm Hg, P=0.043). Contrary, no differences in mean IOP values between GC-free group and individuals treated with combined GC and immunosuppressive therapy were detected (P=0.563). Similarly, mean IOP in GC was 17.14±3.94 mm Hg and in GC-free patients was equal to 16.67±3.45 mm Hg (P=0.671). According to treatment regimen no statistical differences in optic disk SD-OCT for RNFL thickness, RNFL symmetry, cupping volume and the C/D ratio were observed. Similarly, no statistical differences for the mean and minimal ganglion cell layer (GCL) thickness measured in macular SD-OCT or NFI in GDx were detected. CONCLUSION: Combined immunosuppressive and systemic GC therapy in SLE patients may lower the risk of iatrogenic ocular hypertension. No relationship between treatment regimen and glaucomatous damage of optic nerve fibers in analyzed groups with SLE is detected.

LUPUS ANTICOAGULANT HYPOPROTHROMBINEMIA SYNDROME: A CASE REPORT

Lupus anticoagulant hypoprothrombinemia síndrome (LAHPS) is a rare entity. A 54-year-old woman diagnosed with systemic lupus erythematosus (SLE) present in August 2020 with cerebellar haemorrhage, coagulation factor II deficiency was found. After increasing corticosteroid dose and adjustment of immunosuppressive therapy FII levels increased. She has no presented new haemorrhagic events.

On the Nose: Anti-MDA-5 Dermatomyositis Manifesting as Perinasal Swelling

The clinical presentation of dermatomyositis (DM) is diverse, with varied phenotypes that may be correlated with specific autoantibodies. The anti-melanoma differentiation-associated gene 5 (MDA5) antibody in DM is associated with an amyopathic phenotype of DM, with several unusual cutaneous manifestation and increased risk for rapidly progressive interstitial lung disease. Initial presentation may be subtle, but early diagnosis is key to initiation of proper immunosuppressive therapy. In this report, we describe perinasal edema and erythema as a presenting complaint of anti-MDA5 DM in an otherwise healthy 40-year-old woman. The edema began shortly after heavy sun exposure and was followed by painful papules in her hands and arthritis within a few weeks. She was found to have high titer of anti-CCP and anti-MDA5, and thus was diagnosed with DM and rheumatoid arthritis overlap. A CT chest, abdomen, and pelvis showed patchy ground-glass and interstitial opacities in bilateral lower lobes consistent with mild interstitial lung disease without evidence of malignancy. Perinasal cutaneous findings and arthralgias improved with initiation of prednisone. To our knowledge, this is the first report of perinasal edema as a presenting symptom for DM and should raise suspicion for MDA-5 disease.

Cost of illness of oral lichen planus: a multicenter university hospital–based outpatient observational study

Objectives To estimate the economic costs of oral lichen planus (OLP) through a multicenter university hospital–based outpatient study conducted in Italy and Finland. Materials and methods A multicenter retrospective study was conducted on patients affected by OLP to evaluate the economic cost of managing the disease. Direct costs concerning diagnostic procedures, therapeutic management, and follow-up visits were obtained from clinical records. Statistics was performed with IBM SPSS Statistics. Results One hundred and eight patients with a confirmed diagnosis of OLP (81 women and 27 men), 58 Italians and 50 Finnish, were enrolled in this study. The mean annual cost was 1087.2 euros per patient. The mean annual cost was higher in Finnish than in Italian cohort (1558.7 euros vs. 680.7 euros—p < 0.05). Within the Italian cohort, the local immunosuppressive therapy group and atrophic and erosive OLP type had a higher cost (p < 0.05). Within the Finnish cohort, the local immunosuppressive therapy group had a higher cost (p < 0.05). Conclusions OLP-related costs are very similar to other chronic oral disorders (i.e., periodontitis) with differences between investigated countries. Moreover, patients with more severe clinical features, who need immunosuppressive therapy, are facing more expensive costs. Clinical relevance. In this multicenter cost of illness study, we estimated the direct health care costs of OLP and we found that patients with more severe clinical features, who therefore need symptomatic therapy, are facing more expensive costs.

ECG Changes Through Immunosuppressive Therapy Indicate Cardiac Abnormality in Anti-MDA5 Antibody-Positive Clinically Amyopathic Dermatomyositis

Anti-melanoma differentiation-associated gene 5 (MDA5) antibody, a dermatomyositis (DM)-specific antibody, is strongly associated with interstitial lung disease (ILD). Patients with idiopathic inflammatory myopathy (IIM) who are anti-MDA5 antibody positive [anti-MDA5 (+)] often experience chest symptoms during the active disease phase. These symptoms are primarily explained by respiratory failure; nevertheless, cardiac involvement can also be symptomatic. Thus, the aim of this study was to investigate cardiac involvement in anti-MDA5 (+) DM. A total of 63 patients with IIM who underwent electrocardiography (ECG) and ultrasound cardiography (UCG) during the active disease phase from 2016 to 2021 [anti-MDA5 (+) group, n = 21; anti-MDA5-negative (-) group, n = 42] were enrolled in the study, and their clinical charts were retrospectively reviewed. The ECG and UCG findings were compared between the anti-MDA5 (+) and anti-MDA5 (-) groups. All anti-MDA5 (+) patients had DM with ILD. The anti-MDA5 (+) group showed more frequent skin ulcerations and lower levels of leukocytes, muscle enzymes, and electrolytes (Na, K, Cl, and Ca) than the anti-MDA5 (-) group. According to the ECG findings obtained during the active disease phase, the T wave amplitudes were significantly lower for the anti-MDA5 (+) group than for the anti-MDA5 (-) group (I, II, and V4–6 lead; p &lt; 0.01; aVF and V3, p &lt; 0.05). However, the lower amplitudes were restored during the remission phase. Except for the E wave, A wave and Sep e’, the UCG results showed no significant differences between the groups. Four patients with anti-MDA5 (+) DM had many leads with lower T wave and cardiac abnormalities (heart failure, diastolic dysfunction, myocarditis) on and after admission. Though anti-MDA5 (+) patients clinically improved after immunosuppressive therapy, some of their ECG findings did not fully recover in remission phase. In conclusion, anti-MDA5 (+) DM appears to show cardiac involvement (electrical activity and function) during the active phase. Further studies are necessary to clarify the actual cardiac condition and mechanism of these findings in patients with anti-MDA5 (+) DM.

Clinical Characteristics and Efficacy of Adalimumab and Low-Dose Methotrexate Combination Therapy in Patients With Vogt–Koyanagi–Harada Disease

This retrospective study investigated the clinical characteristics and efficacy of adalimumab and low-dose methotrexate combination therapy in patients with Vogt–Koyanagi–Harada disease who were treated at Hiroshima University from February 2012 to May 2021. The patients' demographics, clinical features at administration of immunosuppressive therapy, steroid-sparing immunosuppressive therapy, side effects, and relapses were recorded. The efficacies of steroid-sparing immunosuppressive therapy (methotrexate, cyclosporine A, adalimumab, and adalimumab and methotrexate combination therapy) were analyzed. Among 62 patients, the median age at diagnosis was 47 years and the median duration of uveitis was 51 months. Systemic corticosteroid therapy was administered to 93.5% of patients (n = 58). Thirty-four patients (54.8%) were treated with steroid-sparing immunosuppressive therapy. Methotrexate and cyclosporine A were administered to 12 and 22 patients, respectively; relapse occurred in 50.0% and 22.7% of the patients, respectively. Discontinuation of cyclosporine A was required in 63.6% of patients because of side effects. Adalimumab was administered to 14 patients. Recurrence occurred in 11 patients, requiring methotrexate concomitantly. The mean dose of methotrexate at inflammatory quiescence after side effect-related dose decrease was 8.0 mg/week (0.13 mg/kg). The median duration of combination therapy without recurrence was 20 months. There were no serious adverse events during adalimumab therapy. A high relapse rate was observed in patients receiving methotrexate; a high rate of side effects requiring discontinuation was observed in patients receiving Cyclosporine A. Patients with late-stage Vogt–Koyanagi–Harada disease may achieve better control with adalimumab and methotrexate combination therapy.

A Case of Clinically Amyopathic Dermatomyositis that was Refractory to Intensive Immunosuppressive Therapy including Tofacitinib, but Successfully Treated with Plasma Exchange Therapy

ABSTRACT Clinically amyopathic dermatomyositis (CADM) patients often develop rapidly progressive interstitial lung disease (RP-ILD). A high level of anti-melanoma differentiation-associated gene 5 antibodies (anti-MDA5 Ab) before treatment is associated with RP-ILD development, a poor treatment response, and poor survival. The prognosis of CADM patients remains poor due to ILD even with combined intensive immunosuppressive therapy. Recently, several additional therapies, including tofacitinib (TOF) and plasma exchange (PE) therapy, have been reported to be effective. We herein report a case of CADM-ILD with a high level of anti-MDA5 Ab that was refractory to combined intensive immunosuppressive therapy including TOF, but successfully treated with PE. The following are possible reasons why TOF was ineffective: 1) cytokines that were not suppressed by TOF played an important role in RP-ILD; 2) TOF was administered later than previously reported; and 3) TOF did not suppress pathological substances such as antibodies. On the other hand, PE removes cytokines and various pathological substances. Therefore, PE may be a more reasonable additional therapy for intractable CADM-ILD.