Combination Of Oral Corticosteroids Research Articles

Punctate Inner Choroidopathy (PIC) disease recurrence with inflammatory choroidal neovascular membrane (iCNVM) post-COVID-19 vaccine.

To report a recurrence of punctate inner choroidopathy (PIC) with an inflammatory choroidal neovascular membrane (iCNVM) after the Pfizer-BioNTech COVID-19 vaccine. Case report. A 38-year-old female with a history of myopia and previous episodes of PIC and iCNVM presented with distorted vision in her right eye, seven days after receiving the first dose of the Pfizer-BioNTech COVID-19 vaccine. The patient exhibited active PIC lesions with iCNVM confirmed on multimodal imaging. Treatment with a combination of oral corticosteroids and intravitreal anti-VEGF injection led to disease resolution. Subsequent COVID-19 vaccinations, administered while the patient was immunosuppressed, did not lead to disease relapse. However, relapse occurred following the fourth COVID-19 vaccine, when the patient was not immune suppressed. This case highlights the potential risk of PIC disease relapse following COVID-19 vaccination. Further research is needed to investigate the relationship between COVID-19 vaccination and PIC exacerbation, as well as to determine optimal management strategies in this population, including close observation and consideration of prophylactic immune suppression at the time of COVID-19 vaccine for high-risk individuals.

Haploinsufficiency of A20 caused by a novel pathogenic missense variant of TNFAIP3 and successfully treated with anti-TNF and immunosuppressive therapies

Loss-of-function of protein A20, encoded by TNFAIP3, leads to an early-onset haploinsufficiency of A20 (HA20). This study reports one Chinese child with HA20 and explores the genetic etiology of TNFAIP3 variant. The patient exhibited transient recurrent episodes of fever, intermittent signs of arthritis, gastrointestinal symptoms and multiple colonic ulcers. Laboratory tests revealed elevated inflammatory indicators and mild to moderate anemia. Genetic analysis identified a heterozygous de novo variant in his TNFAIP3 gene (c.740C＞T, p. P247L), which had never been reported before. The novel missense variation was validated to be pathogenic through causing insufficient expression of A20, over-activation of NF-κB signaling pathway and elevated levels of proinflammatory cytokines in response to stimulation by lipopolysaccharide. A combination of oral corticosteroids, TNF-α inhibitors and thalidomide freed him from symptoms and abnormal inflammatory indicators. Furthermore, continual improvement of the patient's condition was observed during a follow-up period of five months. We demonstrate a case with a de novo missense variant resulting in a loss-of-function of TNFAIP3, which expands the clinical spectrum of HA20. Cytokine antagonists and immunosuppressants may be effective drugs.

The comparation of different oral corticosteroids withdrawal methods for nasal polyp surgery.

To compare the efficacy and adverse events of different oral corticosteroids (OCSs) withdrawal methods for chronic rhinosinusitis with nasal polyp after endoscopic sinus surgery (ESS). This was a randomized prospective study conducted from Oct 2019 to Jan, 2021. 35 patients who underwent ESS were randomly divided into 2 groups. Regular group (n = 18) received 0.4mg/kg/day of methylprednisolone orally for 10 days, tapering group (n = 17) received 0.4mg/kg/day of methylprednisolone orally for 7 days, followed by progressive reduction of 8 mg of methylprednisolone per week for 3 weeks. The visual analogue scale (VAS) score, the Lund-Kennedy endoscopy (LKE) score, and the E score were assessed preoperatively and at half and 1, 2, and 12 months postoperatively. Statistical analyses were performed using SPSS. There was no statistical difference in the baseline characteristics between the 2 groups. The postoperative VAS scores and LKE scores of patients were significantly improved from those preoperatively (P-values < 0.05). There was no statistical difference in the LKE score, E score, and VAS score between the 2 groups both preoperatively and postoperatively (P-values > 0.05). There was no statistical difference in adverse events between the 2 groups (P-values > 0.05). The combination of OCSs and ESS can improve the clinical symptoms of patients and the recovery of nasal mucosa. There was no difference between the 2 drug withdrawal methods in efficacy and adverse events. Drug withdrawal gradually is more complicated therefore, in clinical practice, OCSs withdrawal directly would be the better option for patients.

Long-term follow-up of a combined rituximab and cyclophosphamide regimen in renal anti-neutrophil cytoplasm antibody-associated vasculitis.

ABSTRACTBackgroundCurrent guidelines advise that rituximab or cyclophosphamide should be used for the treatment of organ-threatening disease in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), although few studies have examined the efficacy and safety of these agents in combination.MethodsWe conducted a single-centre cohort study of 66 patients treated with a combination of oral corticosteroids, rituximab and low-dose pulsed intravenous cyclophosphamide followed by a maintenance regimen of azathioprine and tapered steroid for the treatment of biopsy-proven renal involvement in AAV. Patients were followed for a median of 56 months. Case–control analysis with 198 propensity-matched cases from European Vasculitis Study Group (EUVAS) trials compared long-term differences in relapse-free, renal and patient survival.ResultsAt entry, the median Birmingham Vasculitis Activity Score (BVAS) was 19 and estimated glomerular filtration rate was 25 mL/min. Cumulative doses of rituximab, cyclophosphamide and corticosteroids were 2, 3 and 4.2 g, respectively, at 6 months. A total of 94% of patients achieved disease remission by 6 months (BVAS < 0) and patient and renal survival were 84 and 95%, respectively, at 5 years. A total of 84% achieved ANCA-negative status and 57% remained B cell deplete at 2 years, which was associated with low rates of major relapse (15% at 5 years). The serious infection rate during long-term follow-up was 1.24 per 10 patient-years. Treatment with this regimen was associated with a reduced risk of death {hazard ratio [HR] 0.29 [95% confidence interval (CI) 0.125–0.675], P = 0.004}, progression to end-stage renal disease (ESRD) [HR 0.20 (95% CI 0.06–0.65), P = 0.007] and relapse [HR 0.49 (95% CI 0.25–0.97), P = 0.04] compared with propensity-matched patients enrolled in EUVAS trials.ConclusionsThis regimen is potentially superior to current standards of care, and controlled studies are warranted to establish the utility of combination drug approaches in the treatment of AAV.

Behçet's disease in black skin. A retrospective study of 50 cases in Dakar.

Although Behçet's disease is well-documented in Eastern populations, epidemiologic data in Sub-Saharan African population is scarce. The aim of this study was to define the epidemiologic and therapeutic aspects as well as clinical course of Behçet's disease in African black population. The study included 50 patients with Behçet's disease. The average age was 32 (18-67) years. A total of 31 patients were men and 19 were women. Two patients had a positive family history of Behçet's disease. The oral and genital aphthous lesions were present in 100% of patients. The pathergy test was positive in 16 patients (32%). Following skin conditions were observed: pseudofolliculitis in 15 patients (30%), acneiform papules in 6 patients (12%), erythema nodosum in 4 patients (8%) and leg ulcers in one patient. Ocular involvement was reported in 22 patients (44%) and joint involvement in 20 patients (40%). Neurological abnormalities were noted in 12 patients (24%). Gastrointestinal involvement with wide and deep ulcerations in the ileocecal region was observed in a patient. As treatment, a combination of oral corticosteroids and colchicine was used in 97% of our patients. Thalidomide was introduced in 3 patients and anticoagulation treatment in 19 patients. Clinical improvement was noted in 25 patients (50%), recurrence in 14 patients (28%) and 3 patients were lost to follow (6%). The Behçet's disease is not uncommon in black skin and generally affects young adults. Severe aphthous ulcers of the oral cavity and genital area are the most consistent finding.

Dermatomiositis. Aportación de un caso de afectación leve-moderada y disfagia precoz

We report the case of a twelve year old female patient with juvenile dermatomyositis. The diagnosis was made in our unit a few days after starting with proximal muscular weakness and a skin rash. A combination of oral corticosteroids and methotrexate was administered. There was an initial improvent in the skin lesions, but with no changes in the muscle weakness. The appearance of dysphagia a few days after starting the treatment led us to add three pulses of parenteral methylprednisolone to her initial treatment. We report the case to illustrate the difficulties in deciding initial treatment options.

Combination of Oral Corticosteroids and Polidocanol Sclerotherapy in the Management of Infantile Hemangiomas

Infantile hemangiomas (IHs) are the most common benign tumors of infancy and may need to be treated in some cases. Oral corticosteroids are the mainstay of therapy but are not without their attendant side effects. To evaluate the efficacy of combination therapy with oral corticosteroids and polidocanol sclerotherapy in hemangiomas needing treatment. Eight children with IHs were started on oral prednisolone 3 to 4 mg/kg per day and fortnightly sclerotherapy with 3% polidocanol solution. The outcome was evaluated on the basis of color, turgidity of the lesion, ongoing growth, and assessment by parents and physician. Serial photographic records were maintained. The response was graded as excellent, good, poor, or nil. Complications were recorded. Four male and four female patients presented at a mean age of 4.6 months. The average area of the lesions was 40.6 cm(2) . The mean duration of treatment with steroids at full dose was 8.2 weeks and mean total duration of treatment was 28.6 weeks. Mean number of sclerotherapy sessions required was 4.7. Complications included ischemic necrosis, rebound growth, and ulceration. Combining oral corticosteroids with polidocanol sclerotherapy produced excellent results in the patients treated, with minimal attendant side effects.

Periocular xanthogranuloma: A forgotten entity?

Periocular xanthogranulomatous diseases are a rare group of disorders which are characterized by a predilection to affect the orbit and ocular adnexa and special histopathological features, in particular infiltrates comprising non-Langerhans-derived foamy histiocytes and Touton giant cells. The differential diagnosis is difficult and occasionally definite diagnosis cannot be established even after clinical and histopathological findings are taken together. We describe a case of a middle-aged man who presented with a 10-year history of voluminous eyelid swelling with concomitant late-onset atopic manifestations, namely bronchial asthma and allergic rhinitis with nasal polyps. After thorough clinical and laboratory investigation, including a biopsy of the eyelid, we classified the patient's disease to a rare entity that has been relatively recently described: periocular xanthogranuloma associated with adult-onset asthma. In a review of the literature, no prospective trials concerning the treatment of this disease were found. The literature mainly contained case reports and case series in which corticosteroids and chemotherapy with alkylating agents have been reported to be beneficial. We treated our patient with a combination of oral corticosteroids and cyclophosphamide pulses and we observed substantial regression of the eyelid masses together with a normalization of systemic immunologic abnormalities.

Allergic Bronchopulmonary Aspergillosis, Allergic Aspergillus Sinusitis and their Co-occurrence

Allergic bronchopulmonary aspergillosis (ABPA), which requires a set of criteria for diagnosis, occurs in atopic individuals, predominantly asthmatics. Oral corticosteroids are the cornerstone for the management of the disease. Allergic Aspergillus sinusitis (AAS), clinico-pathologically similar to ABPA, is also diagnosed with a set of criteria including demonstration of fungal elements in sinus material. Heterogeneous densities on computed tomography of the para-nasal sinuses are caused by the ‘allergic mucin’ in the sinuses. A combination of oral corticosteroids and surgical removal of impacted sinus mucin is the current approach to treatment. Despite common clinico-immunopathological characteristics, the co-occurrence of both these diseases is a rarely reported phenomenon. This could be due to the fact that the two diseases are often encountered by different specialities. Screening all asthmatics for Aspergillus sensitisation could identify those with severe disease and those at risk for developing ABPA. AAS must be excluded in all patients with ABPA and vice-versa.

Successful response to rituximab in two cases of acquired haemophilia refractory to standard-therapy

Acquired autoantibodies against coagulation factors (acquired haemophilia) frequently constitute a life-threatening bleeding situation requiring a prompt therapeutic intervention, including control of bleeding and secondarily an attempt of eradication of the inhibitor by prolonged immunosuppressive therapy. The combination of oral corticosteroids and cyplophosphamide seems to be effective to eradicate the autoantibody, but some patients may be resistant. Another therapeutic approach, recently described, observes treatment with the chimeric anti-CD20 monoclonal antibody rituximab. We report two consecutively treated patients whose acquired FVIII inhibitors did not respond to standard immunosuppressive regimens, and only when rituximab was added to therapy, complete response and prolonged remission were obtained.

Acquired haemophilia: management of bleeds and immune therapy to eradicate autoantibodies

Acquired haemophilia is a rare, but often severe bleeding disorder caused by autoantibodies against a coagulation factor, usually factor VIII (FVIII). Between 1997 and 2004 we observed 14 patients (mean age of 78 years) with acquired haemophilia. The aim of the present study was to investigate the effect of activated prothrombin complex concentrate (aPCC) for bleeds and the response to corticosteroids and cyclophosphamide to eradicate the offending autoantibodies. The most common clinical presentations were severe profuse bruising (12) and haematuria (5). Ten patients were classified as idiopathic. At the time of diagnosis all patients had a very low FVIII level, and one patient also showed factor IX < 1%. High levels of antibodies to FVIII varying from 10 to 1340 Bethesda units (BU) and prolonged activated partial thromboplastin time were disclosed in all patients. Eight severe bleeds were treated with aPCC (FEIBA) at a dosage of 70 IU kg(-1) every 8 h until haemostasis. Ten patients received corticosteroids and cyclophosphamide as immunomodulatory therapy. Effective haemostasis was achieved in all bleeds after aPCC. Ten of 11 patients responded either completely or partially to the immunomodulatory regime within 6 months. Five patients achieved complete response (CR) whereas partial responses were seen in five patients. The anti-CD20 monoclonal antibody rituximab was given to two patients in conventional doses and a CR was seen in one patient. aPCC is effective in treating acute bleeds in patients with acquired haemophilia with high inhibitor levels. The combination of oral corticosteroids and cyclophosphamide seems to be effective to eradicate the inhibitor.

Peripapillary choroidal neovascularisation in sarcoidosis

Purpose : To describe a rare manifestation of sarcoidosis. Methods : Case report of a patient with histologically proven sarcoidosis, who developed peripapillary choroidal neovascularisation in the absence of uveitis or optic nerve disease. Results : Oral corticosteroids achieved reduction in the size of the peripapillary choroidal neovascularisation. Laser treatment was effective in treating the remaining peripapillary choroidal neovascularisation, resulting in improvement of visual acuity. Conclusions : Isolated peripapillary choroidal neovascularisation is a previously unreported complication of sarcoidosis. A combination of oral corticosteroids and laser can be successful in treating this type of lesion, thereby preventing permanent visual loss.

Identifying general practice patients diagnosed with asthma and their exacerbation episodes from prescribing data.

To determine the reliability of identifying patients diagnosed with asthma in general practice and their asthma exacerbation episodes from prescribing data. Automated database from 17 general practitioners (29,805 patients) in the northern Netherlands. Sensitivity, specificity and predictive values of four criteria for identifying patients diagnosed with asthma and two criteria for identifying asthma exacerbation episodes were calculated using the registered diagnosis as gold standard. Prescription of one or more anti-asthma medications identified 95% of patients with an asthma diagnosis (positive predictive value 0.70), while two or more anti-asthma medications identified 71% (positive predictive value 0.79). A combination of oral corticosteroids or antibiotics identified 55% of exacerbations. Asthma patients can be identified reliably from prescribing data, but identification of asthma exacerbations was poor. The preference for one criterion over another for identifying patients diagnosed with asthma will depend on the reason for patient selection.

Current Therapy of Chronic Liver Disease

The study of chronic liver disease has been hampered by insufficient information relative to the pathogenesis of the many forms of hepatitis. Consequently, well-designed treatment strategies are frequently lacking. Wilson's disease is characterised by excessive copper accumulation in the liver and other organs. While d-penicillamine is clearly effective, many patients may not tolerate its many adverse effects. Trientine, oral zinc and unithiol have all shown promise as therapeutic alternatives. Autoimmune chronic active hepatitis responds well to prednisone and azathioprine. Cyclosporin has also produced clinical improvement in several case reports but no comparison has yet been made with the current standard therapy. Recombinant interferon-alpha (IFN alpha) has demonstrated the ability to inhibit hepatitis B viral replication, and the combination of oral corticosteroids followed by IFN alpha is more effective than either agent alone in eliminating viral replication in patients with chronic active hepatitis B. Currently, primary sclerosing cholangitis (PSC) has no standard medical management, but corticosteroids and methotrexate may each have a future role in its treatment. Drug treatment for primary biliary cirrhosis (PBC) has been disappointing, and early reports of success with d-penicillamine were not confirmed in large well-controlled trials. While some reports of improvement with several agents have been described, larger studies are still needed. Alcoholic liver disease continues to be associated with significant morbidity and mortality and numerous investigators have researched several different medical avenues of treatment. Success reported with androgens and the antithyroid agent propylthiouracil in alcoholic liver disease will need confirmation by other research before these agents can be recommended for routine use. Finally, colchicine may prove to be effective in slowing the rate of fibrosis in cirrhosis, but this has yet to be conclusively proven.