Low-dose Prednisolone Research Articles

再発性の脂腺炎に対し低用量プレドニゾロン療法が有効であった秋田犬の1例

再発性の脂腺炎に対し低用量プレドニゾロン療法が有効であった秋田犬の1例

Efficacy of Intravenous Cyclophosphamide Therapy for Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica (NMO) is an inflammatory disease that affects the optic nerve and spinal cord. Optic neuritis and longitudinally extensive myelitis associated with systemic autoimmune disease have been recently defined as NMO spectrum disorder (NMOSD). In this study, we report the efficacy of intravenous cyclophosphamide (IVCY) therapy for NMOSD. Four patients diagnosed with NMOSD were enrolled in this study. The expanded disability status scale (EDSS) score was used to evaluate the degree of severity. All of the patients received intravenous methylprednisolone (IVMP; 1 g/day for three days), and two patients also received plasmapheresis (PP). All of the patients were administered IVCY treatment. Anti-AQP4 antibodies were present in the sera of all patients. All patients exhibited longitudinally extensive transverse myelitis (LETM). Only one patient who fulfilled the criteria for a diagnosis of NMO exhibited optic neuritis. Two patients developed relapse under treatment with low-dose prednisolone (PSL) before the administration of IVCY. The patients in this study exhibited a median improvement in the EDSS score following IVCY treatment from 8.0 to 5.75. Adverse effects were observed in only one patient. This study, despite its retrospective design, demonstrated the therapeutic efficacy of IVCY for NMOSD in both the acute and chronic phases of the disease and determined the IVCY dosage for Japanese women with NMOSD. Additionally, this study provided evidence that for NMOSD patients with severe disabilities, IVCY added to IVMP and PP may be a useful therapeutic modality.

Early treatment with addition of low dose prednisolone to methotrexate improves therapeutic outcome in severe psoriatic arthritis

Psoriatic arthritis (PsA) is increasingly being recognized to cause progressive joint damage and disability. PsA unresponsive to non-steroidal anti-inflammatory drugs (NSAIDs), the conventional first-line choice of treatment, is usually managed with disease-modifying antirheumatic drugs (DMARDs) especially methotrexate. An 18-year-old HIV-negative male had progressively severe PsA of 4-month duration that was nearly confining him to a wheel chair. He did not respond to multiple NSAIDs, alone or in combination with methotrexate (15 mg/week), given for 4 weeks. Addition of prednisolone (10 mg on alternate days) controlled his symptoms within a week. The NSAIDs could be withdrawn after 4 weeks as the treatment progressed. The doses were tapered for methotrexate (5 mg/week) and prednisolone (2.5 mg on alternate days) every 8 weekly subsequently during 15 months of follow-up without recurrence/deformities or drug toxicity. For years, the use of corticosteroids in psoriasis has been criticized for their propensity to exacerbate the skin disease on withdrawal. However, monitored use of corticosteroids, even in low doses, combined with DMARDs may be a good therapeutic option in early stage of the PsA rather than ‘steroid rescue’ later. This will help in early control of joint inflammation, prevent joint damage and maintain long-term good functional capacity and quality of life. This may be useful when the cost or availability of biologics precludes their use. However, we discourage the use of corticosteroids as monotherapy.

The evolution of clinicopathologic features in eruptive lichen planus: A case report and review of literature

"Eruptive" or "exanthematous" lichen planus (LP) is a rare variant of lichen planus. Here we report a middle-aged woman with a 6-month history of episodic eruptive LP presenting as generalized, erythematous, flat-topped, round, polygonal, or umbilicated papules and hyperpigmented macules. The disease was under good control with continuous low-dose prednisolone over one year. We had the opportunity to correlate the clinical and pathologic findings based on histopathologic examination of three separate skin lesions that appeared to represent different stages of evolution. Recognition of these 3 chronological manifestations (polygonal papule in active inflammation, centrally umbilicated papule in involution, and hyperpigmented macule in resolution) can aid the diagnosis.

Pulmonary Mycobacterium abscessus disease in a patient receiving low-dose methotrexate for treatment of early rheumatoid arthritis

A 70-year-old woman with methotrexate (MTX)-refractory rheumatoid arthritis (RA) was referred to our hospital for introduction of biological therapy. On high-resolution computed tomography scans, the patient exhibited abnormal findings such as bronchiectasis and centrilobular small nodules, which were highly suggestive of pulmonary nontuberculous mycobacterial (NTM) disease. Although mycobacterial cultures of sputum specimens yielded negative results, cultures of bronchoalveolar lavage fluids grew Mycobacterium abscessus. Frequent follow-up chest radiographs indicated that the patient's pulmonary disease became rapidly worse in 1month following dose escalation of MTX and administration of low-dose prednisolone. Oral clarithromycin and levofloxacin, chosen on the basis of in vitro susceptibility testing, led to a dramatic recovery from this potentially life-threatening complication. Through our experience with this case, we have learned that (1) pulmonary M. abscessus disease can progress rapidly, even during nonbiological anti-RA therapy; (2) regular follow-up chest radiographs are useful to ensure timely implementation of anti-NTM treatment; (3) bronchoscopic testing should be considered when patients are suspected of pulmonary NTM disease but do not meet the diagnostic criteria; and (4) early isolation, identification, and susceptibility testing of causative NTM species are critical for favorable outcomes.

Low-dose prednisolone in early rheumatoid arthritis inhibits collagen type I degradation by matrix metalloproteinases as assessed by serum 1CTP--a possible mechanism for specific inhibition of radiological destruction

To study the effects of low-dose prednisolone on the osteoclast-regulating proteins osteoprotegerin (OPG) and RANK ligand (RANKL) and on markers of bone resorption, 1CTP generated by MMPs and CTX-1 generated by cathepsin K, in patients with early RA in relation to inflammation and joint destruction. In 225 patients, who at the start of the first DMARD had been randomized to 7.5 mg prednisolone daily for 2 years, the P-group, or no prednisolone, the NoP-group, OPG and RANKL were analysed at 0-24 months and 1CTP and CTX-1 at 0-12 months. Radiographs of hands and feet were assessed at 0, 1 and 2 years using the modified Sharp-van der Heijde score and radiological progression defined as increase in total Sharp score above 5.8. Data were analysed with a mixed linear model and by the GENMOD procedure. In the P-group, RANKL and the ratio OPG/RANKL were stable between baseline and 24 months, whereas in the NoP-group, RANKL increased and the ratio OPG/RANKL decreased. CTX-1 decreased significantly more in the P-group. 1CTP decreased over time in both groups, but more in the P-group, P < 0.001, a difference also present in the subgroups of patients in remission. The decrease in 1CTP was associated with less radiological progression after 2 years and displayed a significant interaction with treatment. Low-dose prednisolone may inhibit progression of joint destruction by interfering with MMP activity, seen as a marked decrease in 1CTP, as well as by impairing osteoclast activation, shown by a stable OPG/RANKL ratio.

Ten-year experience of pulsed intravenous cyclophosphamide and methylprednisolone protocol (PICM protocol) in severe ocular inflammatory disease

AimsSevere ocular inflammation is a blinding ophthalmological emergency. This study evaluates the efficacy and patient tolerance of a validated regime of pulsed intravenous cyclophosphamide and methylprednisolone (‘PICM protocol’) for these...

Steroid psychosis in an adrenal insufficiency and hypothyroidism patient

Acute psychosis after corticosteroid treatment is not unusual; however, the induction of psychosis by a low dose of corticosteroid is uncommon. We describe the case of a 77-year-old man who had adrenal insufficiency and Hashimoto's thyroiditis who, with previous corticosteroid exposure, developed insomnia, sexual hallucinations, aggression and exhibited self-harm behavior after the administration of cortisone 25 mg in the morning and 12.5 mg in the evening. These psychiatric symptoms improved when the corticosteroid therapy was discontinued, but recurred after re-administering 5 mg prednisolone per day. Agitation persisted even at a very low dose of prednisolone, namely 2.5 mg per day. He recovered after corticosteroid treatment was stopped.

Correlation of 18F-FDG PET/CT assessments with disease activity and markers of inflammation in patients with early rheumatoid arthritis following the initiation of combination therapy with triple oral antirheumatic drugs

This study evaluated the potential of functional imaging to monitor disease activity and response to treatment with disease-modifying antirheumatic drugs (DMARD) in DMARD-naive patients with early rheumatoid arthritis (RA). The study involved 17 patients with active RA in whom combination therapy was initiated with methotrexate, sulfasalazine, hydroxychloroquine, and low-dose oral prednisolone. Clinical disease activity was assessed at screening, at baseline and after 2, 4, 8 and 12weeks of therapy. (18)F-FDG PET/CT of all joints was performed at baseline and after 2 and 4weeks of therapy. (18)F-FDG maximum standardized uptake values showed a reduction of 22 ± 13% in 76% of patients from baseline to week 2 and a reduction of 29 ± 13% in 81% of patients from baseline to week 4. The percentage decrease in (18)F-FDG uptake from baseline to week 2 correlated with clinical outcome, as measured by the disease activity score (DAS-28) at week 12. In addition, changes in C-reactive protein levels and erythrocyte sedimentation rate were positively associated with changes shown by PET. (18)F-FDG PET/CT findings after 2 and 4weeks of triple combination oral DMARD therapy correlated with treatment efficacy and clinical outcome in patients with early RA. (18)F-FDG PET/CT may help predict the therapeutic response to novel drug treatments.

Treatment of refractory obstetric antiphospholipid syndrome: the state of the art and new trends in the therapeutic management

To date, there are no reliable data regarding the actual treatment received by women with refractory obstetric antiphospholipid syndrome (OAPS). The aim of this study was to assess current clinical evidence and new trends in the treatment of refractory OAPS. A non-systematic but comprehensive literature search using relevant keywords was made to identify relevant articles published in English from different computerized databases: PubMed (Medline), Google Scholar electronic database search and The Cochrane Library, from January 2000 to March 2012. Studies on the treatment of poor obstetric outcomes in women with OAPS were included. Prospective randomized clinical trials, cohort studies, reviews, systematic reviews and meta-analysis were retrieved. A total of 130 articles were finally selected for this review, including 17 randomized clinical trials and four meta-analyses. The majority of articles were non-randomized original papers and basic and clinical reviews. Up to 20% of women with OAPS do not receive the currently recommended therapeutic regimen. Unfortunately, well-designed studies regarding the usefulness of new drugs in refractory OAPS are scarce. Hydroxychloroquine and low-dose prednisolone appear to be useful when added to standard therapy. Current data do not support the use of intravenous immunoglobulins in this field. The role played by double anti-aggregant therapy, fondaparinux, vitamin D, pentoxifylline and TNF-targeted therapies should be tested in well-designed studies.

Nontyphoid Salmonella-infected mycotic aneurysm in a patient with rheumatoid arthritis

A 60-year-old male presented, and was admitted, to our hospital with a 1-week history of intermittent chills and high fever. He has rheumatoid arthritis for 2 years with regular medication of hydroxychloroquine, methotrexate, sulfasalazine, and low-dose prednisolone (5 mg daily). The patient had a history of type 2 diabetes for which he was under regular medication. General malaise and dyspnea would occur during the episode of fever. On admission to our hospital, his blood pressure was 129/74 mm Hg, and his body temperature was 39 C. His heart sounds were normal with no murmur; the lungs were clear to auscultation. The abdomen was soft without palpable masses or tenderness; the liver and spleen were not palpable. The patient had mild back pain. A complete blood count revealed 9,000 leukocytes/lL and 221,000 platelets/uL, with mild anemia (hemoglobin: 9.4 g/dL). The C-reactive protein (CRP) level was 24.1 mg/dL (normal\0.5 mg/dL) and the erythrocyte sedimentation rate (ESR), 64 mm/h (normal \15 mm/h). Urine analysis and culture yielded negative results. Two blood cultures obtained before intravenous antibiotic administration tested positive for salmonella group D. No vegetations or signs of infective endocarditis were detected on transthoracic echocardiography. Computed tomography (CT) showed a saccular aneurysm arising from the abdominal aorta, with fatty stranding, free air-bubbles (arrow indicates air-bubbles and asterisk indicates aneurysm; Fig. 1) and periaortic fluid accumulation (Fig. 2). Mycotic aneurysm was confirmed. The patient received surgical management for debridement of abscess and intravenous ceftriaxone 2 gm daily continuously for 8 weeks. The patient had an excellent clinical response and was discharged 2 months later with no recurrence of fever. Mycotic aneurysms are uncommon of all aortic aneurysms, and the overall mortality is about 50 % after treatment. The common initial symptoms include pain over abdomen or back, fever, general discomfort, and hypovolaemic shock [1]. The most frequent site of mycotic aneurysms is abdominal aorta, followed by the thoracic and suprarenal abdominal aorta. High level of ESR and CRP can be found from the laboratory data. Abdominal CT can be used for early detection of mycotic aneurysms. Saccular, multilobulate, periaortic soft tissue stranding, and irregular peripheral enhancement of arterial walls can be found from the image study. The cultures from blood or the aneurismal content include Salmonella species, staphylococcus aureus, Streptococcus pneumoniae, and Mycobacterium tuberculosis [1]. Immunocompromised patients including HIV infection, patients with received immunosuppressive agent may develop fulminating bacteremia, which leads to the formation of infected aneurysms [2, 3]. Antimicrobial treatment alone usually results in poor prognosis; therefore, surgery may play a major role in reducing mortality. The combination of antimicrobial treatment and surgical debridement of surrounding necrotic tissue with revascularization is indicated in the patient with mycotic aneurysm. D.-H. Yang (&) Division of Rheumatology/Immunology/Allergy, Department of Internal Medicine, Taichung Armed Forces General Hospital, No 348, Sec. 2, Chung Shan Road, Taiping 411, Taichung, Taiwan, Republic of China e-mail: deng6263@ms71.hinet.net

Effective intravenous immunoglobulin therapy for Churg-Strauss syndrome (allergic granulomatous angiitis) complicated by neuropathy of the eighth cranial nerve: a case report

IntroductionWe report the case of a patient with Churg-Strauss syndrome with eighth cranial nerve palsy. Vestibulocochlear nerve palsy is extremely rare in Churg-Strauss syndrome. To the best of our knowledge, only one case of complicated neuropathy of the eighth cranial nerve has been described in a previous report presenting an aggregate calculation, but no differentiation between polyarteritis nodosa and Churg-Strauss syndrome was made. High-dose immunoglobulin was administered to our patient, and her neuropathy of the eighth cranial nerve showed improvement.Case presentationAt the age of 46, a Japanese woman developed Churg-Strauss syndrome that later became stable with low-dose prednisolone treatment. At the age of 52, she developed sudden difficulty of hearing in her left ear, persistent severe rotary vertigo, and mononeuritis multiplex. At admission, bilateral perceptive deafness of about 80dB and eosinophilia of 4123/μL in peripheral blood were found. A diagnosis of cranial neuropathy of the eighth cranial nerve associated with exacerbated Churg-Strauss syndrome was made. Although high doses of steroid therapy alleviated the inflammatory symptoms and markers, the vertigo and bilateral hearing loss remained. Addition of a high-dose immunoglobulin finally resulted in marked alleviation of the symptoms associated with neuropathy of the eighth cranial nerve.ConclusionsA high dose of immunoglobulin therapy shows favorable effects in neuropathy of the eighth cranial nerve, but no reports regarding its efficacy in cranial neuropathy have been published.

A randomized, double-blind, placebo-controlled trial of low-dose oral prednisolone for treating painful hand osteoarthritis

Anti-inflammatory therapies are effective analgesics for OA. This study determined whether low-dose oral prednisolone (PNL) was an effective analgesic for hand OA. This was a randomized, double-blind, placebo-controlled trial of people with ACR criteria hand OA and baseline hand pain visual analogue scale (VAS) of >40/100 mm. Participants received 5 mg PNL or placebo daily for 4 weeks. Pain VAS, disease activity VAS, Australian/Canadian Hand Osteoarthritis Index and joint counts were performed at baseline, 4 and 12 weeks. Primary outcome was the change in hand pain VAS at 4 weeks. Analysis of covariance was used for analysis, controlling for baseline values. To explore potential mechanism of action of PNL, non-contrast 0.2 Tesla MRI was performed on the most painful hand at baseline and 4 weeks. A total of 70 participants were recruited (57 women, mean age 61 years, mean baseline pain VAS 61.5 mm); 75% had more than one joint with definite MRI synovitis/effusion. At 4 weeks the adjusted mean reduction in pain VAS was 19.9 mm (PNL group) and 16.8 mm (placebo group) (P = 0.54). There were no statistically significant differences in VAS, Australian/Canadian Hand Osteoarthritis Index or joint counts between placebo and PNL groups at 4 or 12 weeks. A total of 20 participants in each group achieved an Outcome Measures in Rheumatology-Osteoarthritis Research Society International response. Baseline synovitis/effusion did not predict response to treatment. This is the first randomized controlled trial of low-dose corticosteroid alone for painful hand OA, which demonstrated that short-term low-dose oral PNL is not an effective analgesic treatment for hand OA. International Standard Randomised Controlled Trial Number Register, www.isrctn.org, Trial number 99697616.

Effects of Short-Course Oral Corticosteroid Therapy in Early Dengue Infection in Vietnamese Patients: A Randomized, Placebo-Controlled Trial

Patients with dengue can experience a variety of serious complications including hypovolemic shock, thrombocytopenia, and bleeding. These problems occur as plasma viremia is resolving and are thought to be immunologically mediated. Early corticosteroid therapy may prevent the development of such complications but could also prolong viral clearance. We performed a randomized, placebo-controlled, blinded trial of low-dose (0.5 mg/kg) or high-dose (2 mg/kg) oral prednisolone therapy for 3 days in Vietnamese patients aged 5-20 years admitted with dengue and fever for ≤72 hours, aiming to assess potential harms from steroid use during the viremic phase. Intention-to-treat analysis was performed using linear trend tests with a range of clinical and virological endpoints specified in advance. In addition to recognized complications of dengue, we focused on the are under the curve for serial plasma viremia measurements and the number of days after enrollment to negative viremia and dengue nonstructural protein 1 status. Between August 2009 and January 2011, 225 participants were randomized to 1 of the 3 treatment arms. Baseline characteristics were similar across the groups. All patients recovered fully and adverse events were infrequent. Aside from a trend toward hyperglycemia in the steroid recipients, we found no association between treatment allocation and any of the predefined clinical, hematological, or virological endpoints. Use of oral prednisolone during the early acute phase of dengue infection was not associated with prolongation of viremia or other adverse effects. Although not powered to assess efficacy, we found no reduction in the development of shock or other recognized complications of dengue virus infection in this study.

Autoimmune hepatitis type 2 associated with an unexpected and transient presence of primary biliary cirrhosis-specific antimitochondrial antibodies: a case study and review of the literature

BackgroundUnlike other autoimmune liver diseases, primary biliary cirrhosis (PBC) has never been reported in early childhood, while type 2 autoimmune hepatitis (AIH) is eminently a paediatric disease.Case presentationWe describe a case of type 2 AIH with serological positivity for PBC-specific anti-mitochondrial antibodies (AMA) in a 3-year old girl. We found this observation intriguing as AMA and indeed an overlap with PBC are virtually absent in Type 2 AIH, a pediatric form of AIH which is distinct precisely because it is characterized by pathognomonic anti-liver kidney microsomal type 1 (LKM-1) showing a remarkable antigen-specificity directed against cytochrome P4502D6. We also review the literature in relation to AMA positivity in paediatric age and adolescence. In our case, the presence of AIH-2-specific anti-LKM-1 and PBC-specific AMA was confirmed by indirect immunofluorescence (IIF), and immunoblotting and ELISA based on recombinant mitochondrial antigens. The clinical, laboratory and histological features of the child are given in detail. Interestingly the mother was AMA positive without other features of PBC. The child was successfully treated with immunosuppression and five years after the original diagnosis is on a low dose of prednisolone and azathioprine, with no signs of relapse. Anti-LKM-1 antibodies are still present in low titres. AMA were detectable for the first 4 years after the diagnosis and disappeared later.ConclusionThis is the first case report in the literature of AIH type 2 with an unexpected PBC-specific AMA positivity in a young child. Response to immunosuppressive treatment was satisfactory and similar to that described in AIH. A review of published reports on AMA positivity in paediatric age shows that the antibody may arise in the context of immunodeficiency and is variably associated with liver damage.

IgG4-related disease of the infratemporal fossa: A case report

IgG4-related disease is a relatively new disease entity characterized by elevated serum IgG4 levels and marked infiltration of IgG4-positive plasma cells in mass lesions. IgG4-related disease of the head and neck region is often associated with the salivary glands, and Küttner's tumor and Mikulicz's disease of the salivary glands may also be IgG4-related diseases. We encountered a patient with an inflammatory pseudotumor (IPT) of the infratemporal fossa, which was histopathologically confirmed to be IgG4-related disease. The patient was a 53-year-old man who presented with tenderness of the right upper jaw, ocular pain, and trismus for approximately 3 months. Imaging revealed a lesion with unclear borders extending from the infratemporal fossa into the right orbit. Soft tissue tumor was suspected and biopsy was performed under general anesthesia. Histopathological examination revealed plasma cell infiltration and fibrosis. Immunohistochemistry revealed prominence of IgG4-positive plasma cells and confirmed the diagnosis of IgG4-related disease. The patient responded well to steroidal therapy, with pain and trismus resolving. Imaging revealed marked reduction in the size of the mass. The patient is currently under observation on low-dose oral prednisolone with no evidence of relapse.

Tacrolimus without or with the addition of conventional immunosuppressive treatment in juvenile autoimmune hepatitis

To investigate tacrolimus (Tac)-based treatment in juvenile autoimmune hepatitis (AIH). Twenty patients (13 girls; age, 8-17 years; median, 13.25 years) with AIH were treated with two daily oral doses of Tac. Six of them had advanced liver disease and/or cirrhosis. Drug concentrations in blood were measured regularly, and the target trough levels were 2.5-5 ng/mL. The patients were followed up for 1 year. Their clinical, biochemical, immunological and histological status was obtained at baseline and after 1 year. In three cases, Tac alone led to complete remission. In 14 cases, additional low doses of prednisolone or azathioprine were used for a short time to achieve remission. In two cases, the treatment was discontinued: in one because of therapeutic failure, in another because of a suspected but unverified adverse event. Ten patients reported headache and/or recurrent abdominal pain. Two patients developed inflammatory bowel disease. Renal function remained intact. Tac is a promising alternative first line of treatment for AIH. Although monotherapy with Tac is usually not sufficient to achieve complete remission, the prednisolone and azathioprine doses can be drastically reduced, and most of their side effects avoided.

Avances de interés clínico en el diagnóstico y tratamiento de los pacientes con síndrome antifosfolípido

In this paper we review recent studies and consensus documents that we consider relevant to the diagnosis and treatment of patients with antiphospholipid syndrome (APS). The diagnosis of APS is based on the Sydney classification criteria (2006), in which positive laboratory tests (anticardiolipin antibodies, anti-β2-glycoprotein I antibodies or lupus anticoagulant) are mandatory. However, it is not uncommon to see patients with clinical features highly suggestive of the syndrome in whom these antibodies are persistently negative. Therefore, we summarise the principal clinical and serological findings in a subgroup of patients with seronegative APS in the first series published up to date. In addition, a recent study draws attention to the safety and efficacy of the long-term use of low-molecular-weight heparins in patients with APS not susceptible to warfarin treatment. There is also a subgroup of women with APS and recurrent foetal loss with no response to the standard antithrombotic therapy; in this group the materno-foetal prognosis could be improved by the addition of low-dose prednisolone during the first trimester of pregnancy. Finally, we list the principal recommendations regarding thromboprophylaxis in APS drawn from the expert consensus document elaborated at the meeting held in Galvestone (2010).

Extraokuläre Manifestationen des Morbus Behçet

Behcet's disease is a multisystem disorder with the histopathological correlate of leukocytoclastic vasculitis. The classification criteria for the disease include the presence of recurrent oral aphthous ulcers combined with at least two other manifestations, such as genital aphthous ulcers, skin manifestations (mostly erythema nodosa or pseudofolliculitis) and ocular manifestations (panuveitis or posterior uveitis with retinal vasculitis). A positive pathergy test is regarded as pathognomonic for the disease and a sterile papulopustule occurs after a sterile needle prick of the forearm. However, this test is positive in only 15% of the patients. The prognosis of Behcet's disease becomes unfavorable when vital organs are involved. This is the case for involvement of the central nervous system which occurs in 10% of patients, arterial and pulmonary arterial aneurysms and gastrointestinal involvement, which clinically and histopathologically is difficult to differentiate from inflammatory bowel disease but tends to perforate. Oligoarthritis, which occurs in approximately 50% of the patients, causes problems concerning the differential diagnosis from classical forms of spondyloarthritis. Behcet's disease is associated with HLA-B51 in 50-80% of the cases depending on the country of origin of the patient. The prognosis becomes unfavorable if the disease manifests in young male patients. The treatment of extraocular manifestations depends on the aggressiveness. Milder manifestations are treated with low dose prednisolone and steroid sparing immunosuppressants, such as azathioprine or cyclosporine A. In cases with more severe manifestations, such as central nervous system (CNS) involvement cyclophosphamide or TNF antagonists and in selected cases also interferon alpha can be considered.

Complete remission induced by tacrolimus and low-dose prednisolone in adult minimal change nephrotic syndrome: A pilot study

BackgroundFew clinical trials have examined the replacement of steroids with other immunosuppressive drugs as a primary treatment modality for minimal change disease (MCD) in adults. We studied the efficacy of tacrolimus to induce complete remission (CR) in adults with MCD. MethodsWe enrolled 14 adults with MCD and nephrotic-range proteinuria. All patients were treated with oral tacrolimus 0.05mg/kg twice daily and prednisolone 0.5mg/kg/day. CR was defined as a urine protein to creatinine ratio of<0.2g protein/g creatinine (g/g cr). The primary outcome was cumulative percentage of CR during 16 weeks. ResultsThe mean urine protein to creatinine ratio at enrollment was 10.9g/g cr (range: 4.2–18.1g/g cr). The trough tacrolimus level was maintained at 5.99±2.63ng/mL. CR was achieved by 13/14 (92.8%) patients within 8 weeks. The cumulative CR rate was 7.7% (1/14), 64.2% (9/14), 71.3% (10/14), and 92.9% (13/14) at 1 week, 2 weeks, 4 weeks, and 8 weeks, respectively. The one remaining patient achieved CR at 20 weeks after treatment, who was followed up for a further 4 weeks. The mean time to achieve CR in the 14 patients was 4.64±5.11 (1–20) weeks. Three cases suffered adverse events of abdominal pain, diarrhea, or new-onset diabetes mellitus. ConclusionTacrolimus and low-dose prednisolone therapy induced CR rapidly (71.3% by 4 weeks and 100% by 20 weeks) and effectively in adult patients with MCD.