Efficacy and Safety of High-Dose of Mycophenolate Mofetil Compared With Cyclophosphamide Pulse Therapy as Induction Therapy in Japanese Patients with Proliferative Lupus Nephritis

Objective To clarify the effectiveness and safety of induction therapy with mycophenolate mofetil (MMF) in patients with lupus nephritis (LN). Methods Patients with LN administered MMF (n = 35) or IVCY (n = 25) plus high-dose corticosteroids between July 2015 and June 2020 were included. MMF was increased from 2 g/day to 3 g/day, with no adverse events (AEs). The primary endpoint was the 6-month renal remission rate. Secondary endpoints were retention rate and AEs. Results There were no significant differences in age, sex, disease duration, renal histological type, SLEDAI, and UPCR between the two groups. Twenty-six patients (74%) continued with MMF therapy, whereas twelve (48%) completed six IVCY courses. The retention rate was significantly higher in the MMF than in the IVCY group (p = 0.048). Twenty-four and fourteen patients in MMF and IVCY groups, respectively, achieved renal remission with insignificant differences. Grade 3 or higher AEs were observed in eight and fourteen patients in the MMF and IVCY groups, respectively (p = 0.014). Conclusions The efficacy of high-dose MMF was comparable to that of IVCY in Japanese patients with proliferative LN, with fewer AEs and a higher retention rate than IVCY, suggesting the high tolerability of MMF.

Pancreaticoduodenal artery aneurysm associated with polyarteritis nodosa presenting as massive upper gastrointestinal bleeding

The pancreaticoduodenal arteries are rare sites for true aneurysm formation, but these may develop in association with occlusion of the coeliac circulation, degenerative conditions or inflammatory vascular disorders. These have a high risk of rupture regardless of size or other factors. One identified cause is polyarteritis nodosa (PAN), which is an autoimmune necrotising vascular condition that affects small-sized and medium-sized arteries. We report a case of a 40-year-old man with massive gastrointestinal tract bleeding from a ruptured pancreaticoduodenal artery aneurysm secondary to PAN. This was managed with emergent open aneurysm ligation followed by high-dose corticosteroids and cyclophosphamide pulse therapy. Only three other cases of PAN-associated pancreaticoduodenal artery aneurysms have been reported in the literature.

Primary phlebitis of central nervous system revealed by black-blood magnetic resonance imaging

Primary phlebitis of the central nervous system (PPCNS) is a rare condition that might be a subset of primary angiitis of the CNS. In this case report, the patient was a 39-year-old man with a 2-week history of anterograde amnesia and abnormal behaviours. Black-blood MRI (BB-MRI) showed contrast enhancement of the left basilar vein and cerebral superficial veins. Angiography showed unremarkable change in arteries. After a thorough differential diagnosis, we diagnosed PPCNS and then administered methylprednisolone pulse and cyclophosphamide pulse. The neuropsychological symptoms and MRI findings gradually improved, and after 2 months, the dose of prednisolone was gradually reduced to 20 mg. No recurrence was observed. This case shows that BB-MRI may be useful for diagnosing PPCNS.

COST COMPARATIVE ANALYSIS OF DEXAMETHASONE-CYCLOPHOSPHAMIDE PULSE THERAPY AND RITUXIMAB THERAPY IN PEMPHIGUS IN A GOVERNMENT TERTIARY CARE HOSPITAL IN NORTHERN INDIA.

Pemphigus vulgaris is the most common autoimmune blistering disorder and the prevalence varies from 0.18 to 6.96 cases per 1,00,000 population worldwide [3].Pulse therapy with high doses of Corticosterids and Cyclophosphamide (most commonly Dexamethasone Cyclophosphamide Pulse (DCP)) for the treatment of pemphigus remained mainstay therapy for past few decades. Targeted immuno-modulation is becoming popular with advent of biological therapies like Rituximab due to lower side effects but its cost is a limiting factor. There is a little literature available on comparison of cost of these therapies which lead us to compare cost of these therapies. In a country like India where cost is a greater parameter to decide therapy a cost effective therapy carries a better compliance and effectiveness as compared to an expensive therapy like Rituximab. In the study we observed DCPtherapy was three times cheaper than Rituximab

A Case of Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis Overlap Syndrome

An overlap of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibodies- (ANCA-) associated vasculitis (AAV) is extremely rare: approximately 40 cases have been reported to date. A literature review indicates that they are more common in women in their forties, and simultaneous onset has been reported in 69% of cases. In addition, both lupus nephritis and ANCA-associated glomerulonephritis were observed on renal biopsy. This report presents the case of a 35-year-old woman with an 8-month history of polyarthralgia who was admitted to our hospital. She was diagnosed with SLE due to typical clinical presentation of the disease: polyarthritis, lymphocytopenia, hypocomplementemia, presence of antinuclear and anti-dsDNA antibodies, and proteinuria. However, purpura were scattered, and the titer of antimyeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) was high. A skin biopsy revealed leukocytoclastic vasculitis that involved poor immune complex deposition. A renal biopsy showed necrotizing glomerulonephritis with cellular and fibrocellular crescent formation that involved deposition of IgM and C3c only in the mesangial area and the peripheral capillaries. Additionally, no electron-dense deposits were observed under electron microscopy. These pathological findings were consistent with AAV rather than with SLE. Therefore, we finally diagnosed the patient with both SLE and microscopic polyangiitis. After treatment with methylprednisolone and intravenous cyclophosphamide pulse therapies, renal function improved and MPO-ANCA levels decreased. In cases of suspected overlap between SLE and AAV, appropriate diagnosis and treatment are important.

Safety and Efficacy Evaluation of Traditional Chinese Medicine (Qingre-Lishi-Yishen Formula) Based on Treatment of Regular Glucocorticoid Combined with Cyclophosphamide Pulse in Children Suffered from Moderately Severe Henoch–Schonlein Purpura Nephritis with Nephrotic Proteinuria

Objective. At present, the most appropriate management of Henoch–Schonlein purpura nephritis (HSPN) with nephrotic-range proteinuria still remains controversial; thus, the purpose of this study is to evaluate safety and efficacy of traditional Chinese medicine (TCM), Qingre-Lishi-Yishen Formula (QLYF), integrated with regular oral glucocorticoid and cyclophosphamide intravenous pulse therapeutic regimen in children suffered from moderately severe HSPN with nephrotic proteinuria. Methods. From 1 January 2012, to 1 January 2016, totally 150 hospitalized children suffered from HSPN with nephrotic proteinuria were included. All were treated with glucocorticoid and cyclophosphamide, and 100 of them were treated with integrative traditional Chinese decoction QLYF. Patients were followed up for 2 years. Rate of adverse event occurrence, short-term clinical effects, long-term clinical effects, and TCM therapeutic evaluation were all compared. Results. Total adverse event rate was lower in the QLYF group (χ2 = 5.357, p=0.022); rates of respiratory infection, urinary infection, poor appetite, hepatotoxity, cardiotoxicity, and neutropenia were all decreased in patients who received QLYF (p<0.05), and no cases of hepatic and renal toxicities related to the herbal medicine were observed in the QLYF group. For short-term clinical efficacy evaluation, lower levels of 24 hour proteinuria (p<0.05) and urine blood cell count (p<0.05) were found in the QLYF group. For long-term efficacy evaluation, better clinical control rate effective rate, lower recurrence rate (p<0.05), and fewer TCM syndrome score (p<0.05) were found in the QLYF group. Conclusion. Compared with merely using regular oral glucocorticoid plus cyclophosphamide pulse therapeutic regimen, the therapeutic regimen that integrates QLYF with the abovementioned western medicine might be a safe means to decrease the occurrence rate of adverse events and improve short-term and long-term clinical effects in children who suffered from moderately severe HSPN with nephrotic proteinuria.