Low-dose Prednisone Research Articles

A rare case of Polyarteritis Nodosa associated with autoimmune hepatitis: a case report

BackgroundPolyarteritis nodosa is a type of vasculitis affecting medium- and small-sized arteries that has been associated with hepatitis B but does not have an established relationship with autoimmune hepatitis. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa.Case presentationA 45-year-old woman was diagnosed with autoimmune hepatitis after initially presenting with a two-month history of fatigue, nausea, and anorexia and a three-week history of scleral icterus. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. Azathioprine was discontinued and she was treated with pulse steroids followed by a prednisone taper, cyclophosphamide, and intravenous immune globulin with overall improvement in her symptomatology. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil.ConclusionsIn patients with recent diagnosis of autoimmune hepatitis, there should be a modest suspicion for concomitant polyarteritis nodosa if symptoms and signs of multisystem vasculitis develop.

Role of immunosuppressive therapy in the management refractory postprocedural pericarditis.

To assess the safety and efficacy of a novel immunosuppressive regimen-combination Methotrexate/Prednisone (cMtx/P)-in the management of severe refractory rPPP. In this multicenter, nonrandomized, retrospective, observational study, 408 consecutive patients diagnosed with persistent rPPP between 2017 and 19 were included. Patients with refractory symptoms despite 3 months of conventional therapy were initiated on a 4-week regimen of oral steroids. Persistence of symptoms at this point, that is, rPPP (n = 25; catheter based = 18, open surgical = 7) prompted therapy with Methotrexate (7.5-15 mg weekly) with folate supplementation along with low dose prednisone (5 mg PO) for a further 3 months. Patients were followed for a total of 11.3 ± 1.8 months. Treatment refractory rPPP occurred in 6.1% of the study population prompting immunosuppressive therapy with cMtx/P. All patients demonstrated complete symptom resolution following 3 months of treatment with an 85% decline in clinically significant pericardial effusions. One patient developed recurrent pericarditis during the 11-month follow-up. Therapy was well tolerated with no significant drug related adverse effects. cMtx/P therapy is a safe and effective adjunct in the management of rPPP refractory to standard therapy.

Pyoderma Gangrenosum-Like Presentation of Herpetic Panniculitis in a Patient With Immunosuppression

Introduction. This case of herpes virus–induced panniculitis originally diagnosed as pyoderma gangrenosum (PG) illustrates the need for a high index of suspicion for atypical causes of cutaneous ulcers in patients who are immunocompromised. Case Report. A 79-year-old male presented with a 3-month history of a painless chronic ulcer on the left buttock that was refractory to antibiotic therapy and intralesional corticosteroid. The medical history was notable for diabetes mellitus type 2 and rheumatoid arthritis managed with long-term methotrexate and low-dose prednisone. Because the patient initially had a painful and enlarging skin ulcer after intralesional treatment with corticosteroids, an undermined and violaceous ulcer, and an autoimmune condition, PG was suspected at the initial evaluation. A subsequent skin biopsy to complete the workup confirmed the unexpected diagnosis of herpetic panniculitis. The patient was started on antiviral therapy; a prolonged therapeutic and suppressive dose was required. This case highlights the importance of skin biopsy in the diagnosis of chronic ulcers to rule out infectious etiologies. Maintaining a high index of suspicion for rare causes of cutaneous ulcers in the patient with immunosuppression is paramount. Conclusions. Herpes virus infection is only one atypical cause of ulcerative nodules in the immunocompromised patient. Skin biopsy should be considered in the immunocompromised patient with presumed PG that is not responding to standard of care treatment.

Low-Dose Prednisone Therapy is Efficacious in Treating Painful Subacute Thyroiditis

Subacute thyroiditis (SAT) usually presents with neck pain, radiating to ears and is often associated with hyperthyroidism. Currently the available treatment involves administration of NSAID or in more symptomatic patients prednisone 40mg daily tapered over 6 weeks or longer. We report successful treatment of 3 patients (Pts) with SAT with low-dose prednisone therapy (20mg/day) (LDP20) initially and tapered over 4 weeks. Patient 1: A 32-year-old female presented with severe neck pain radiating to both ears and low- grade fever of 2-weeks duration. Two weeks prior, patient had cold-like symptoms lasting for 3 days. Physical examination: HR 110bpm, tremors of fingers noted, tenderness of the anterior neck present, thyroid 30-gms in size. Labs: ESR 92 mm/hr, CRP 3.2 mg/dL, TSH <0.005 uIU/mL, free T4 2.71 ng/dL, total T3 168 ng/mL. Thyroid scan and uptake showed a 24-hrs uptake <1%, thyroid gland not visualized, consistent with SAT. Patient was treated with atenolol and LDP20 tapered over 4 weeks. Pain significantly improved after 2 days of treatment. Six weeks later TSH was 0.9 uIU/mL with a free T4 1.4 ng/dL and ESR 8 mm/hr. Patient 2: A 19-year-old female presented with left-ear pain, anterior neck pain, fever, and extreme fatigue. PE: HR 111bpm, heat shield present, tender-to-palpation thyroid, brisk DTR. Lab: CBC normal, ESR 98 mm/hr, CRP 9.9 mg/dL, TSH <0.01 uIU/mL, free T4 3.8 ng/dL, total T3 210 ng/mL. Thyroid scan and uptake: uptake <1%, no thyroid gland visualized and SAT was diagnosed. Patient was started on LDP20 and atenolol. Four days following prednisone therapy her symptoms completely resolved and prednisone was tapered off over 4 weeks. Thyroid functions were normal by the seventh week. Patient 3: A 38-year-old male presented with fever, fatigue, severe neck pain, palpitation and a weight loss of 8 pounds. PE: HR 120 bpm, thyroid severely tender on palpation, brisk DTR. Lab: normal CBC, ESR 128 mm/hr, CRP 11.9 mg/dL, TSH <0.001 uIU/mL, free T4 4.2 ng/dL, total T3 201 ng/mL. Thyroid scan: thyroid gland not visualized and uptake was < 1%. SAT was diagnosed and patient was treated with propranolol and LDP20. After 5 days the dose of prednisone was reduced to 15mg/day and the prednisone was tapered over five weeks. Patient had resolution of symptoms in 70 hours and remained asymptomatic for the next 12 months of follow-up. Thyroid function normalized by the eighth week. Conclusion: SAT is a painful disabling thyroid disorder apparently caused by a viral infection; and NSAID or high-dose steroid treatment remains the standard of care. We have treated 3 Pts with relatively lower doses of prednisone than previously recommended and attained remission successfully. Thus side effects can be avoided with lower prednisone dose.

Virilization Secondary to an Ovarian Leydig Cell Tumor

A 60-year-old female presented with a three-year history of virilizing symptoms including facial hirsutism and deepening of voice. Her medical history was significant for renal transplantation with immunosuppressive therapy consisting of mycophenolate, cyclosporine, and low-dose prednisone. She was noted to have temporal balding and darkly pigmented terminal hair on the upper lip, cheeks, chin, shoulders, and sternum. Pelvic examination revealed clitoromegaly. Menarche occurred at age 12 with regular menstrual cycles until menopause which occurred at age 50. She had two pregnancies: a miscarriage followed by a successful pregnancy.Labs revealed an elevated total testosterone of 530 ng/dL (< 60 ng/dL), free testosterone 14.8 ng/dL (<0.87 ng/dL), androstenedione 2140 ng/dL (<200 ng/dL), and 17-hydroxyprogesterone 704 ng/dL (<285 ng/dL). LH, FSH, and estradiol were inappropriately normal in this post-menopausal female. Prolactin, TSH, DHEA-S, IGF-1 were within normal limits. Transvaginal ultrasound found a 2 cm hypoechoic right ovarian mass which was confirmed on MRI. MRI also revealed a 5 mm right adrenal nodule. Tumor markers including CA-125, Inhibin A, Inhibin B, HCG, and AFP were within normal limits. Dexamethasone suppression testing did not lower the testosterone level. 17-hydroxyprogesterone level after cosyntropin stimulation testing was 704 ng/dL (<1000 ng/dL). The patient underwent laparoscopic bilateral oophorectomy and salpingectomy, pelvic washout and omental biopsy. Pathology was consistent with a benign Leydig cell tumor. Following oophorectomy there was complete normalization of the total testosterone level (15 ng/dL, n< 60 ng/dL).A thorough history and physical exam is vital in determining the cause of hirsutism. Medications, including over-the-counter and herbal formulations should be carefully reviewed. Although cyclosporine has been associated with hirsutism, patients typically present with vellus hair formation in the affected areas rather than darkly pigmented terminal hair. In this case, hirsutism progressively worsened following menopause and physical examination was significant for virilization. Hirsutism in a combination with virilization is typically neoplastic in nature. Endogenous androgen production can originate from either the adrenal glands or ovaries. In our patient, with workup showing both ovarian and adrenal as potential sources of endogenous androgen production, an adrenal cause was excluded due to a normal DHEA-S level at baseline and a lack of suppression of testosterone after dexamethasone suppression testing. As a result, the source was localized to the ovary. While excessive androgen production resulting in virilization is seen with ovarian tumors, Leydig stromal cell tumors are extremely rare and account for less than 0.1% of all ovarian tumors.

Patch Testing During Immunosuppressive Therapy: A Systematic Review.

Patch testing, used in the assessment of allergic contact dermatitis, is ideally avoided in patients receiving immunosuppressive therapy because of concerns with reductions in accuracy; however, this is not well characterized in the literature. This systematic review summarizes patch testing results in patients receiving immunosuppressive therapy. We identified 16 studies, comprising 195 patients with dermatitis or psoriasis, who were patch tested while receiving immunosuppressants. Of these, 7 studies, comprising 85 patients with dermatitis, patch tests were performed before and during immunosuppression. Overall, 67.9% (n = 19) of the dermatitis patients receiving dupilumab maintained positive reactions to an allergen that previously graded as a 2+/3+ reaction. Several immunosuppressants were also associated with positive patch test results for various allergens. These include dupilumab, cyclosporine, and low-dose prednisone (≤10 mg/d) for dermatitis, and tumor necrosis factor α inhibitors, ustekinumab, and methotrexate for psoriasis. Ideally, it is preferable to patch test when patients are not receiving oral immunosuppressants or immunomodulators. However, clinicians may choose to assess the risks and benefits of patch testing for each patient given the impact of allergic contact dermatitis on patient quality of life.

Calcitriol-mediated hypercalcemia as an immune-related adverse event in a patient receiving nivolumab and ipilimumab for metastatic renal cell carcinoma, case report

BackgroundSevere hypercalcemia is often associated with uncontrolled malignancy through several mechanisms. However, calcitriol-mediated hypercalcemia is a rare etiology for advanced solid tumors.Case presentationWe report a case of calcitriol-mediated hypercalcemia secondary to immune checkpoint inhibition in a responder with metastatic clear cell renal cell carcinoma (ccRCC). In this case, a 68 year old male with metastatic ccRCC to the liver within 4 months of right radical nephrectomy went on to develop hypercalcemia (12.8 mg/dL) shortly following 2 cycles of nivolumab and ipilimumab. Additional testing showed an elevated calcitriol level (142 pg/mL), low parathyroid hormone (PTH) and parathyroid hormone-related protein (PTHrP) levels, and a normal 25-hydroxyvitamin D level. FDG-PET imaging showed hypermetabolic mediastinal, hilar, and intra-abdominal lymphadenopathy, however the subsequent lymph node biopsy only showed reactive lymphoid cells without malignancy or granuloma. The hypercalcemia was resistant to initial therapy with calcitonin, hydration, and zoledronic acid but quickly responded to high-dose prednisone (1 mg/kg), followed by normalization of calcitriol levels. The patient was rechallenged with nivolumab and ipilimumab which provided a partial response after 4 cycles. He was maintained on low dose prednisone (10 mg daily) leading to a sustained resolution of his hypercalcemia.ConclusionThis case suggests calcitriol-mediated hypercalcemia as a novel immune-related adverse event.

POS-448 CORTICOSTEROID SPARING AGENTS IN FREQUENT RELAPSING AND STEROID DEPENDENT NEPHROTIC SYNDROME IN CHILDREN: A SINGLE CENTER RETROSPECTIVE STUDY

Children with frequently relapsing nephrotic syndrome (FRNS) or Steroid-dependent nephrotic syndrome (SDNS) may be prescribed non-corticosteroid immunosuppressive agents whenever there is a failure to maintain remission with low-dose alternate-day prednisone and/or significant adverse effects of prednisone develop. A wide variety of immunosuppressive agents have been used in these patients to reduce the number of relapses and maintain remission. A retrospective study was conducted on all steroid-sensitive nephrotic syndrome (SSNS) children (1-11 years) who received any type of second line agents (e.g. CNI, MMF, cyclophosphamide, and Rituximab) over a period of 9 years from January 2010 to January 2019 in pediatric nephrology unit in Prince Sultan Military Medical City, Riyadh. The study included 24 patients. Their age at diagnosis ranged between 1 and 11 years with a mean of 3.8 years and standard deviation of (±) 2.6 years. During the first year of steroid therapy, relapse occurred among 87% of patients; of them, the number of relapses being 4 or more in 21.7%. Regarding indication for the second line of treatment, SDNS was the most frequent reported (60.9%), followed by FRNS (30.4%). Concerning agents used in the second line, MMF ranked first (58.4%), followed by Cyclophosphamide (33.3%). Number of relapses after starting steroid sparing agent was more than once among 41.7% of patients. Duration of remission after starting steroid sparing agent ranged between 2 and 72 months (14±14.1). Overall response to the second line of treatment was observed among majority of patients (91.7%). Renal biopsy was performed in 45.8% of patients. Concerning side effects of steroid sparing agents, electrolytes disturbances and hypertension were reported by two (8.3%) and one (4.2%) patients respectively. Duration of remission was significantly longer among patients treated with cyclosporine (48±33.9 months) compared to other lines of treatment, p<0.001. On the other hand, hypertension was only reported among patients treated with cyclosporine, p=0.003. The overall response of children with SDNS and FRNS to the second line agents was significant, with favorable longer remission free period with Cyclosporine use with no major side effects. Our results affected by the retrospective design of the study, as well as the small sample size. Therefore larger scale study with prospective design is highly encouraged.

Granular Cell Tumor in the Brain of a Dog

Diagnostic Exercise from The Latin Comparative Pathology Group. Clinical History: 10 year-old, female spayed, Golden Retriever/Poodle mix. This patient had a mass removed laparoscopically from the right adrenal gland (cortical adenoma) and was started on a low dose of prednisone post-operatively. Eight days post-op, she began having cluster seizures and was started on levetiracetam therapy. The following day, the dog was moderately obtunded and stumbling. On neurological examination, mild generalized ataxia was noted along with decreased menace OS, and delayed proprioception in the left pelvic and left thoracic limbs. CBC and chemistry values were unremarkable except for a mild hepatic enzyme elevation. EEG showed seizure-like activity and abnormal brain waves resembling sleep state while awake. MRI revealed an eccentric right-sided mass extending over the frontal and parietal lobes with subtentorial herniation. Due to poor prognosis, euthanasia was elected. Necropsy and Microscopic Findings: Tenuously adhered to the right parietal lobe of the brain and the dura mater is a soft, round, white, plaque-like mass measuring 3.5 x 3.0 x 0.3 cm. The mass is friable and poorly-demarcated from surrounding brain parenchyma. A portion of the mass adheres to the supradjacent surface of the calvarium.

Calcineurin Inhibitor Sparing Immunosuppression in a 12-year-old Heart Transplant Recipient with a Single Kidney

Introduction Renal dysfunction requires renal transplant or dialysis in up to 10% of transplant patients at 15 years. Those with chronic renal disease or single kidney are at high risk, but there has been limited study of calcineurin inhibitor (CNI)-sparing heart transplantation (HT) in pediatrics. Case Report A 12 year old female with history of Wilms tumor status post left nephrectomy developed anthracycline-induced dilated cardiomyopathy. She had decompensated heart failure bridged to transplant with milrinone. Given prior recurrent acute kidney injury (AKI) in the setting of a single kidney, she was at increased risk of receiving dialysis post-operatively, but a combined heart-kidney transplant was not felt indicated. She was induced with thymoglobulin and initially managed with concomitant mycophenolate mofetil and methylprednisolone. At approximately 3 weeks post-transplant, sirolimus was added. Within 2 week postoperatively, she developed hypertension, acute tubular necrosis, and oliguric AKI. Prior to starting Sirolimus, she also developed pleural and pericardial effusions requiring a chest tube, pericardiocentesis, and pericardial window. Despite these complications there was no evidence of transplant rejection or graft reperfusion injury on biopsy, but filling pressures were upwards of 25 mmHg bilaterally. Aggressive hypertensive management and judicious diuretic use improved her filling pressures and overall clinical status. She was discharged home 60 days after HT. At her 3 month post-op catheterization she had normal filling pressures and no signs of rejection (ISHLT grade A0, AMR grade 0) on low dose prednisone, mycophenolate, and sirolimus. Her AKI has resolved and she remains dialysis free. Summary AKI after HT is due to a constellation of renal hypoperfusion, low cardiac output, and drug-induced nephrotoxicity requiring dialysis in some patients. Prior studies showed improved renal function in those converted from CNIs to mTOR inhibitors. In our case, the morbidity associated with AKI was deemed too severe to pursue standard use of CNIs. While some pediatric patients have undergone immunosuppression with reduced CNI dosing, few have completed HT without CNI. Her case illustrates the utility of mTOR inhibitors for its nephrotoxic sparing profile while maintaining adequate immunosuppression protection and graft function.

Dueling Malignancies: A Rare Instance of Both Kaposi Sarcoma and Cardiac Plasmacytoma in a Patient Post OHT

<h3>Introduction</h3> Judicious immunosuppressive therapy is important to balance immune tolerance of the donor organ, while minimizing the risk of infection and malignancy. This case highlights the development of two unusual malignancies post orthotopic heart transplant (OHT). <h3>Case Report</h3> A 69 year old male, with a history of ischemic cardiomyopathy and no prior history of hematological malignancies underwent OHT. His post-transplant course was complicated by multiple infectious complications, leading to discontinuation of mycophenalate mofetil and he was maintained on an immunosuppressive regimen of tacrolimus and low-dose prednisone. Two years post OHT he noticed the development of a nodule on his 2nd right toe. Biopsy revealed Kaposi Sarcoma (KS). The patient underwent a total body PET scan and was found to have bilateral hypermetabolic inguinal lymph node involvement confirmed as KS by biopsy. His tacrolimus goal was lowered and he was treated with 8 cycles of paclitaxel, which resulted in complete PET resolution. One year later as part of a routine endomyocardial biopsy the patient was found to have a population of plasma cells with kappa restriction that were EBV negative. A TTE showed a structurally normal heart without evidence of mass/tumor. Magnetic resonance imaging showed late gadolinium enhancement in the inferior, basal inferolateral and anterolateral segments. Workup for MGUS/Multiple Myeloma was unrevealing. The diagnosis was thought to be a solitary cardiac plasmacytoma. The decision was made to further minimize immunosuppression to single agent tacrolimus with a goal tac level of 4-6. <h3>Summary</h3> Kaposi Sarcoma and cardiac plasmacyotoma are infrequent to rare tumors encountered in the transplant population. The literature on treatment for patients who develop malignancy post transplantation is sparse, with the majority of anecdotal evidence suggesting that reducing the level of baseline transplant immunosuppression is adequate for resolution of the underlying malignancy.

Management of Cardiac Sarcoidosis after Orthotopic Heart Transplant: A Multi-Institutional Experience

<h3>Purpose</h3> Cardiac sarcoidosis (CS) is a chronic granulomatous disease of uncertain etiology that has the potential to recur in transplanted hearts. Practice guidelines outlining the optimal care of CS patients do not currently exist for heart transplant recipients. We therefore conducted an online survey of heart transplant providers to gain insight into current practice patterns. <h3>Methods</h3> An online survey was distributed to members of the International Society for Heart and Lung Transplantation. It consisted of 16 questions designed to classify provider type, location and management preferences. Differences between groups were analyzed for significance using Chi-square and/or Fisher exact tests. <h3>Results</h3> 82 providers from 49 different transplant centers responded to our survey. Among respondents, 56 (69%) were cardiologists, 11 (13.1%) advanced practice providers, and 3 (3.6%) surgeons. A significantly greater percentage of centers maintained their CS versus non-CS patients on lifelong prednisone (58.2% vs 4.9%, p<0.001). Most selected low dose (prednisone 1-5 mg daily) vs higher or high dose prednisone (67.5% vs 9.9% vs 0%). The majority of responders (60.3%) routinely monitored for recurrence of CS beyond their center-specific period of performing protocol biopsies. For responders that chose to routinely screen for CS recurrence with more than one modality allowed), 17 (19.3%) utilized cardiac MRI and 25 (28.4%) utilized cardiac PET (Figure A). <h3>Conclusion</h3> A significantly greater proportion of CS patients are maintained on lifelong prednisone post-OHT compared to non-CS patients. For routine monitoring of CS recurrence, cardiac MRI and PET appear to be the preferred modalities for assessing CS recurrence in the allograft. However, a significant percentage of providers do not routinely monitor for CS recurrence post-OHT.

Systematic Review of Safety and Efficacy of COVID-19 Vaccines in Patients With Kidney Disease

Systematic Review of Safety and Efficacy of COVID-19 Vaccines in Patients With Kidney Disease

Low-Dose Methotrexate Use in Idiopathic Granulomatous Mastitis: An Alternative Treatment Method

Background: Idiopathic granulomatous mastitis (IGM) is a rare, recurrent and progressive breast disease with an unknown etiology. Patients with IGM will probably face stressful, time-consuming treatment procedures with side effects due to medications. There are different treatment modalities in clinical use including medical and surgical interventions. Objective: The aim of this study was to present the results of using the combination therapy of low-dose methotrexate (MTX) and steroid in IGM. Methods: Seventeen patients diagnosed with IGM and treated with MTX were included into the study. Low-dose MTX at 5 mg/week and 8 mg/day prednisone were given for 2–3 months. Results: After 2–3 months of treatment, 10 patients exhibited (58.5%) complete, 3 patients (17.6%) partial recovery, and no response to the treatment process was observed in 4 patients (23.5%). No side effects of MTX and recurrent events were noted in any of the patients. Conclusion: Low-dose MTX and prednisone treatment for IGM patients, who did not respond to steroids alone, should be considered as an alternative treatment method instead of surgical intervention.

Leflunomide plus low-dose prednisone in patients with progressive IgA nephropathy: a multicenter, prospective, randomized, open-labeled, and controlled trial

Background Immunoglobulin A nephropathy (IgAN) is the most common cause of glomerulonephritis worldwide, and the optimal approach to its treatment remains a significant challenge. Methods We did a prospective, randomized, open-labeled, multicenter, controlled trial, comprised of 3-month run-in, 12-month treatment, and 12-month follow-up phases. After 3-month run-in phase, patients with biopsy-confirmed IgAN at risk of progression were randomly allocated to LEF plus low-dose prednisone (LEF + prednisone group) or conventionally accepted-dose prednisone [prednisone(alone) group] Our primary outcome was 24-h urine protein excretion (UPE) and secondary outcomes were serum albumin (sALB), serum creatinine (Scr), and eGFR. Safety was evaluated in all patients who received the trial medications. Results One hundred and eight patients [59 in LEF + prednisone group, 49 in prednisone (alone) group]were enrolled and finished their treatment and follow-up periods. There is no significant difference in the baseline level between the two groups. Compared with baseline, both groups showed a significant decrease in 24-h UPE (p < 0.01) and increase in sALB (p < 0.01), with stable Scr and eGFR throughout the 12-month treatment period. What’s more, these effects were sustained through the 12-month follow-up period. However, there was no difference in 24-h UPE, sALB, Scr, and eGFR between the two groups (p > 0.05). At 12 months, a difference in overall response rate, relapsing rate, and incidence of adverse events between the two groups was not significant. Conclusions The efficacy and safety of LEF plus low-dose prednisone and conventionally accepted-dose prednisone in the treatment of progressive IgAN are comparable.

Successful Long-Term Control of the Syndrome of Episodic Angioedema With Eosinophilia (Gleich Syndrome) With Low-Dose Imatinib Mesylate and Prednisone.

The syndrome of episodic angioedema with eosinophilia, first reported over 40 years ago, is a hypereosinophilic disorder that, uniquely, is not associated with end-organ pathology. However, patients develop a constellation of symptoms that include angioedema, urticaria, fatigue, and fever. Episodes are accompanied by massive hypereosinophilia and weight gain. Type II serum cytokine levels (IL-5, IL-13, IL-9, and IL-10) show cyclic variations peaking at or just prior to the peak of eosinophilia and an abnormal Th2 cell phenotype has been reported. Attacks may occur with predictable regularity and have been described in both adults and children. Glucocorticoid therapy reliably reverses symptoms with accompanying diuresis, defervesce, and normalization of the eosinophil count. In this report, a patient who had the syndrome of episodic angioedema with eosinophilia exceeding 20 years is reported. He has had no end-organ damage to date. Testing for the CHIC2 deletion, a surrogate for the FIP1L1-PDGFRA fusion, was negative. Use of imatinib mesylate, initially as a steroid-sparing agent, and subsequently as a maintenance medication, plus low-dose prednisone has provided long-term control of hypereosinophilia and all clinical manifestations.

INCIDENCE OF CYTOMEGALOVIRUS DISEASE IN KIDNEY TRANSPLANT RECIPIENTS RECEIVING EVEROLIMUS AND REDUCED TACROLIMUS DOSES: A COHORT RETROSPECTIVE ANALYSIS

Introduction: Cytomegalovirus (CMV) is the most common viral pathogen occurring in postrenal transplantation. CMV infection usually develops during the first few months after transplantation. The success of CMV prophylaxis with antivirals has resulted in a decrease in the incidence of CMV infection. However, CMV remains a significant pathogen, associated with allograft rejection and loss, mortality, interstitial fibrosis and tubular atrophy (IF/TA) in protocol biopsies, and increased post-transplant costs. Purpose: To determine the incidence of cytomegalovirus disease in kidney transplant recipients receiving Everolimus and reduced Tacrolimus doses. Material and Methods: All low immunological risk patients, >18 years old whom received kidney transplantation at Santa Casa de Misericordia de Juiz de Fora between January 2013 and February 2017 were retrospectively assessed. The first group received induction therapy with Basiliximab, and the post-operative maintenance regimen included Tacrolimus, Mycophenolic acid and Prednisone (BAS/MPS), and the second group received Polyclonal antilymphocyte globulins (2,25mg/kg) as induction therapy, and then low dose of Tacrolimus (2mg/10kg/day), Everolimus and Prednisone as maintenance regimen (r-ATG/EVR). None of the patients received pharmacological prophylaxis or preemptive therapy against CMV. Results: Patients receiving EVR showed a lower incidence of CMV when compared to those receiving MPS (4.2% x 17.5%, p=0.005). There was an important difference in the time of hospitalization to treat CMV disease. Patients of the MPS stayed hospitalized for about 20 days more than the EVR group (p=0.005). There was no difference as to the incidence of rejection, delayed graft function or graft survival. Conclusion: Results from this trial conducted in low immunological risk kidney transplant recipients receiving no CMV prophylaxis demonstrated that EVR was associated to a decrease in CMV disease incidence when compared to MPS. These data suggest that kidney transplant recipients receiving EVR may not need CMV prophylaxis.

Observation on the clinical effect of high-dose Intravenous Immunoglobulin combined with low-dose prednisone acetate in the treatment of patients with Kawasaki Disease.

Objective:To evaluate the clinical effect of high-dose intravenous immunoglobulin (HDIVIG) single dose and pulse therapy combined with small-dose prednisone acetate in the treatment of patients with Kawasaki disease (KD).Methods:Eighty patients with KD from Baoding Children’s Hospital, China, were randomly divided into two groups: the experimental group and the control group, each with 40 cases. Patients in the experimental group were treated with HDIVIG single dose, pulse therapy combined with low-dose prednisone acetate, while patients in the control group were treated with conventional-dose immunoglobulin. Patients in both groups were treated with aspirin orally, and given symptomatic treatment including anti-inflammatory, nutritional support, correction of water and electrolyte disturbance and acid-base balance. Peripheral venous blood samples were drawn from all patients at the time of admission, Day-1, Day-7 and Day-14 after treatment, and in the basic state of getting up in the morning, and then the levels of tumor necrosis factor (TNF-a), C-reactive protein (CRP), interleukin-6 (IL-6) and other inflammatory factors were detected by enzyme-linked immunosorbent assay (ELISA). The time of body temperature falling to normal, lymph node swelling recovery, hands and feet swelling, mucosal hyperemia regression after treatment in the two groups was recorded, and the treatment effect of the two groups was comprehensively evaluated.Results:After treatment, the levels of inflammatory factors such as TNF-a, CRP, IL-6 in the experimental group were significantly lower than those in the control group, with a statistically significant difference (P<0.05). In addition, the time of body temperature falling to normal, lymph node swelling recovery, hands and feet swelling, and mucosal hyperemia regression in the experimental group was significantly shorter than that in the control group (p=0.00). The effective rate of the experimental group was 95% and that of the control group was 80%, with a statistically significant difference (p=0.04).Conclusion:HDIVIG single dose, pulse therapy combined with small-dose prednisone acetate has a favourable therapeutic effect in the treatment of patients with KD, by which the inflammatory factors can be significantly improved, clinical symptoms and weight can be quickly ameliorated, and therapeutic effect can be enhanced.

531. COVID-19 infection outcome in African American Renal Transplant recipients: Detroit Medical Center experience

BackgroundTransplant recipients are more vulnerable to infections including COVID-19, given their co-morbidities and chronic immunosuppression. Most preliminary care series report rapid clinical progression and higher mortality compared to the general population.MethodsRetrospective study at Harper University Hospital - Detroit Medical Center. Twenty-five renal transplant recipients (RTR) presenting consecutively with COVID-19 symptoms and positive NP swab PCR for SARS-CoV2 between 03/01/2020 - 05/01/2020 were included. Data on demographics, clinical presentation, laboratory findings, management and outcomes were collected.ResultsAll 25 patients were hospitalized. Patients had a median age of 56, all African American and deceased donor transplant recipients. Most had hypertension (96%), about half (52%) had diabetes, 64% had pulmonary disease including obstructive sleep apnea, COPD and pulmonary hypertension. Most common presenting symptom was dyspnea (64%), followed by fever and cough (56%) and diarrhea (56%). One-half of patients had multifocal opacities on initial chest x-ray (52%). Immunosuppression with tacrolimus and low dose prednisone was continued, while mycophenolate mofetil was held on admission. Following institution guidelines, hydroxychloroquine was given to 32%, while 48% received both hydroxychloroquine and steroids. Prophylactic anticoagulation was given to 80% of patients and therapeutic coagulation to 8%. Oxygen supplementation given to 60% of patients and one patient required intubation. Three patients (12%) required transfer to the intensive care unit, one expired. At follow-up, treatment with mycophenolate was reintroduced based on resolution of symptoms and laboratory parameters.ConclusionCOVID-19 infected RTR in this small cohort had lower mortality of 4% (n=1) compared to State-wide mortality of 10%. Despite multiple co-morbidities and chronic immunosuppression, our patient cohort had excellent prognosis and lower mortality compared to other series. Exact reasons for this optimal outcome are explored.Disclosures All Authors: No reported disclosures

Ocular myasthenia gravis – How effective is low dose prednisone long term?

IntroductionControversy persists on the best treatment to control ocular myasthenia gravis (OMG) and reduce conversion to generalized myasthenia gravis (GMG). We hypothesized that low dose prednisone could accomplish both in a cohort of OMG patients followed after three years. MethodsWe reviewed the records of 168 patients who presented with OMG. Our study included 103 of the OMG patients who met inclusion criteria, requiring follow up for a minimum of 3 years without disease generalization. Low dose prednisone was defined as ≤7.5 mg per day. The main outcome was having single vision without ptosis blocking vision, measured by binocular single vision (BSV) and upper lid position. We also analyzed late progression to GMG. ResultsOf 87 patients treated with prednisone, chronic low dose prednisone alone restored BSV in 47 patients (46% of all patients) without GMG. Pyridostigmine monotherapy restored BSV in 11/14 patients (11% of all patients). Other immunomodulatory therapy (OIT) was needed in 38 patients (37%). Medical therapy maintained BSV at last evaluation (mean follow up 8.2 ± 5.0 years) in 93 patients (90%). GMG developed in 10 patients (10%) during the follow-up period. ConclusionIn OMG patients who do not generalize before 3 years, chronic long term prednisone at lower doses is moderately effective in maintaining optimum BSV. However, OIT are commonly required in these patients. In these OMG patients receiving prednisone and/or OIT, conversion to GMG after three years of disease is uncommon.