Start Of Steroid Treatment Research Articles

#567 Early application of treatment to patients with IgA nephropathy who were supposed to have steroid regimen predicts good renal outcome

Abstract Background and Aims Progressive deterioration of proteinuria and glomerular filtration rate (GFR) is notified in 20-30% of patients with IgA nephropathy during 20-30 years follow-up period. Popular traditional approach for patients with rapid deterioration of renal function is the immunosuppressive treatment with steroid, which efficacy is still remained in debate. We searched the factors related to renal outcomes among patients presumed to need steroid treatment. Method Among 472 adult patients with IgA nephropathy diagnosed by renal biopsy during 2003-2017 in a tertiary hospital, we selected 86 patients with initiation of steroid treatment for more than 1 months and with dose of more than 1000 mg of prednisolone after renal biopsy. We excluded patients with steroid treatment started one month or more before renal biopsy. The renal outcome was defined as decrease of GFR more than 50% from GFR at renal biopsy, decrease of GFR to less than 15 ml/min/1.73 m2, or a status to need renal replacement therapy during follow-up period after renal biopsy. Results At admission for renal biopsy, estimated GFR by CKD-EPI 2009 equation was 68.5 ± 33.3 ml/min/1.73 m2 and urine protein to creatinine ratio (UPCR) was 2.81 ± 2.23 g/g creatinine (no missing data). The renin-angiotensin-aldosterone inhibitor was used in 80 patients (93.0 %) after renal biopsy. Steroid was started at 8.4 ± 23.1 months after renal biopsy. Majority of patients (63 patients) had been prescribed steroid medication within 2 months after renal biopsy. Total dose of steroid was 13.5 ± 13.1 g. The results of eGFR and UPCR before starting steroid medication were 63.7 ± 31.2 ml/min/1.73 m2 and 2.82 ± 2.01 g/g creatinine, respectively. During 63.6 ± 48.8 months of follow-up period after renal biopsy, there were 39 patients (45.3 %) developed the renal outcome. Factors related to development of the renal outcome searched by correlation coefficient were eGFR and UPCR at renal biopsy, medications of anti-diabetic agent, anti-hypertensive agent, immunosuppressive agent other than steroid, dose of steroid, or vitamin D3 agent after renal biopsy, pathologic findings of segmental sclerosis, interstitial inflammation, and tubular atrophy on light microscopic examination, deposition of C3 and IgA on immunofluorescent staining, and Oxford classification of T, period of starting steroid treatment after renal biopsy, and eGFR before starting steroid medication. With Cox's hazard proportional model, the independent factors to predict the renal outcome were eGFR before steroid treatment or eGFR at renal biopsy, immunosuppressive medication other than steroid, pathologic finding of C3 deposition, and period of starting steroid treatment. Hazard ratio to estimate the renal outcome (HR) was 1.025 (95% CI: 1.010-1.039, p = 0.001) for one-month delay of starting steroid medication after renal biopsy. The HR for the renal outcome in patients started steroid medication at 2 months after renal biopsy was 2.353 (95% CI: 1.140-4.856, P = 0.021) compared to patients started steroid within 2 months after renal biopsy. Conclusion For the patients supposed to have indications for steroid treatment at renal biopsy, early initiation of steroid treatment would indicate better renal outcome.

Idiopathic Thrombocytopenia Purpura After Pembrolizumab Treatment Against Locally Recurrent Bladder Cancer : A Case Report

A man in his 70s visited our hospital for gross hematuria. He was diagnosed with invasive urothelial carcinoma (cT3N2M0) and underwent total cystectomy and ileum conduit construction after three courses of neoadjuvant chemotherapy. Eight months after the operation, the disease reoccurred in the pelvic lesion. He received pembrolizumab therapy but developed idiopathic thrombocytopenic purpura (ITP) immediately before the ninth course of administration; and, treatment was discontinued. Recovery of symptoms and normalization of blood test data were achieved 3.5months after starting steroid treatment. Reduction of recurrent disease has been maintained for 2 years.

Importance of eosinophilic infiltration of the colonic mucosa in ulcerative colitis patients who are refractory to maintenance therapy: A prospective, single-center study.

Eosinophilic infiltration is sometimes observed histologically in ulcerative colitis (UC), but the effect of the degree of infiltration on the treatment course for UC is not completely understood. We investigated whether short-term steroid administration in UC patients refractory to maintenance therapy, with high eosinophilic infiltration in the colonic mucosa, contributed to the clinical and endoscopic improvement. Ten patients with endoscopically active and pathologically high eosinophilic infiltration, based on pathological examination using endoscopic biopsy, were examined for the clinical background when starting steroid treatment. The clinical and endoscopic improvement before and after steroid use were assessed prospectively. The average initial steroid dosage and duration of use were 21.0 mg and 102.7 days, respectively. The mean values before and after steroid use of the clinical activity index, the Mayo endoscopic subscore, and the UC endoscopic index of severity were 2.4 and 1.0, 1.8 and 0.7, and 3.9 and 1.1, respectively. All scores improved significantly after steroid use (P = .042, P = .002, P = .002, respectively). Steroids were discontinued in all patients; no patients required steroid re-administration. There may be cases of UC with eosinophilic infiltration into the colonic mucosa and resistance to maintenance treatment, suggesting that short-term steroid administration may contribute to clinical and endoscopic improvements.

Long-Term Growth in Children and Young People with Autoimmune Liver Disease Treated with Daily Steroids.

We aimed to evaluate long-term growth in children and young people with autoimmune liver disease (AILD) treated with daily steroids. This is a retrospective observational cohort study of patients diagnosed between 1992 and 2004 before the age of 16 years. Growth measurements (height, weight and body mass index (BMI)) converted to z-scores were recorded, at diagnosis, 1 and 5 years after commencing treatment and at age 18 years and analyzed together with demographics, disease and treatment related characteristics. Seventy-four patients (35 female) were started on treatment at median age of 12.85 (Inter quartile range (IQR) 9.44, 14.14) years for median duration of 12.07 (IQR 8.68, 13.97) years. At all time-points, the mean z-scores for weight, height and BMI were within the normal range, indicating normal nutritional status. There was no difference in change in z-score for weight, height and BMI from diagnosis until age 18 years when comparing gender (male vs female), ethnicity (Caucasian vs non-Caucasian), diagnosis (AIH vs ASC) and presence of IBD (n = 23). Change in z-score was lower for height and weight for the < 12 years group compared to the ≥12 years age group ( P < 0.05 and P < 0.05, respectively). In addition, change in height z-score correlated positively with age at start of steroid treatment (r = 0.321, P < 0.05) and negatively with duration of steroid treatment (r = -0.321, P < 0.05). Growth of patients with AILD on a daily maintenance dose of steroids remains stable and within normal range during long-term follow up. Small, daily doses are effective in maintaining disease control and minimize the need for high-dose steroid pulses during relapses.

Challenges in the diagnosis of early endogenous fungal endophthalmitis

Endogenous fungal endophthalmitis (EFE) is an uncommon disease caused by the dissemination of fungal infection to posterior segments of the eye and has a high rate of visual loss. Most patients have associated predisposing risk factors, including immunocompromised conditions, and the condition is less likely to occur in healthy individuals. Here, we report two patients who presented like uveitis and deteriorated after starting standard treatment for uveitis, which included steroids. They were later diagnosed with EFE. Fungal endophthalmitis should be suspected even in ambulatory individuals with immunocompromised states, when they present with uveitis like features, before starting steroid treatment. Polymerase chain reaction based diagnosis is extremely useful and should be considered in diagnosing EFE.

Predictive score for oral corticosteroid-induced initial worsening of seropositive generalized myasthenia gravis

BackgroundInitial worsening of symptoms after the start of corticosteroid administration is a major concern in the treatment of myasthenia gravis (MG). However, the risk factors or specific patient backgrounds related to this issue have not been fully understood. We aimed to determine the risk factors and developed a scoring system for predicting initial worsening in generalized MG. MethodsWe enrolled 62 generalized MG patients with anti-acetylcholine receptor antibody. Initial worsening was defined as an increment of three points in the Quantitative MG score within 2 weeks after the start of steroid treatment. A multivariate logistic regression model was used to determine the risk factors, and predictive scores were assigned. Bootstrap resampling was applied to evaluate the risk score model's internal validity. ResultsSteroid-induced initial worsening occurred in 26% of MG patients and was correlated with thymoma-associated or early-onset MG (p = 0.018), initial prednisolone doses ≥40 mg/day (p = 0.029), and upper limb weakness (p = 0.039). Stepwise multivariate logistic regression identified these three clinical factors for predicting initial worsening in MG. A predictive score of 0–3 points had a bootstrapping area under the curve of 0.770 (0.625–0.878). ConclusionsOur scoring system based on three clinical characteristics can predict the likelihood of steroid-induced initial worsening in MG.

Unexpected Visual Loss in Appropriately Treated Giant Cell Arteritis

Unexpected Visual Loss in Appropriately Treated Giant Cell Arteritis

Serum Interleukin 13 Level in Steroid Sensitive Nephrotic Syndrome

Background: There are studies indicating the relationship between interleukin 13 and pathogenesis of nephrotic syndrome. To find this relationship, we measured serum IL13 concentration in acute and remission phases in children with steroid sensitive nephrotic syndrome. Methods: The serum of 15 patients was achieved for measurement of IL-13 in two phases: acute phase and remission phase. To this end, we used Biofarm ELIZA kites. Results: The mean concentration of serum IL-13 was 430 ± 279 ng/mL (227 - 960 ng/mL) before starting steroid treatment. The mean concentration of serum IL-13 was 428 ± 259 ng/mL (215.6 - 960 ng/mL) after 4 weeks of steroid treatment. The difference between pre and post treatment of serum IL-13 levels was not significant (P = 0.91). Conclusions: We showed serum IL13 does not significantly decrease after steroid treatment. Although the role of IL13 in increasing glomerular permeability has been shown in previous studies, the local IL13 may be more important than the systemic form. It also can be postulated that mediators other than IL13 also have primary roles in pathogenesis of proteinuria. However, we need more studies to determine the role of IL13 in the pathogenesis of nephrotic syndrome.

Hypofibrinogenemia associated with steroid therapy for the patients who developed GVHD after allogeneic stem cell transplantation

Hypofibrinogenemia (plasma fibrinogen level <150 mg/dl) is occasionally observed after allogeneic hematopoietic stem cell transplantation, and its etiology is often difficult to determine. We herein report that steroids administered for the treatment of graft-versus-host disease (GVHD) are associated with the development of hypofibrinogenemia. We retrospectively analyzed the plasma fibrinogen (Fg) levels in 15 consecutive patients who had been administered 1 mg/kg/day (1 mg/kg group) or 2 mg/kg/day (2 mg/kg group) methylprednisolone for the treatment of Grade II to IV acute GVHD. Hypofibrinogenemia had developed in 8 of the 15 patients (53%) by day 50 after the start of steroid treatment, and was observed in 2 of 6 patients in the 1 mg/kg group and 6 of 9 in the 2 mg/kg group. A significant decrease in the Fg level was observed in the 2 mg/kg group (the median value before starting steroid treatment and that on the 20th day after starting steroid treatment were 506 mg/dl and 180 mg/dl, respectively, P=0.0013). Other possible causes of hypofibrinogenemia, including liver dysfunction or disseminated intravascular coagulation, were confirmed in only 3 patients during the observation period. In conclusion, hypofibrinogenemia commonly occurs in patients treated with steroids, especially those administered 2 mg/kg/day methylprednisolone for the treatment of GVHD.

SAT0278 The Comparison of Diagnostic Accuracy between Eular/Acr Provisional Classification for PMR and Other Criterion

BackgroundPolymyalgia rheumatica (PMR) is a common rheumatic disorder in the elderly population. General Internal Medicine doctors often encounter these patients. However, the gold standard for the diagnosis of PMR is...

Steroid Use is Associated with Clinically Irrelevant Biopsies in Patients with Suspected Giant Cell Arteritis

Temporal artery biopsy (TAB) is the diagnostic gold standard for giant cell arteritis (GCA). GCA is treated by high-dose corticosteroids. In cases of high clinical suspicion, steroids may be administrated despite negative TAB, making TAB clinically irrelevant. We assessed the role of TAB in clinical decision-making in patients with suspected GCA and to identify factors associated with clinically irrelevant TAB. Charts of patients who underwent TAB from 2005 to 2010 were reviewed for clinical parameters potentially associated with GCA and clinically irrelevant TAB. We studied 143 patients with 99 negative (69%), 34 positive (24%), and 10 undefined (7%) TABs. Eventually 26 patients (18% of the entire cohort and 26% of the patients with a negative TAB) received steroid treatment for GCA despite negative TAB. The start of steroid treatment before TAB was associated with clinically irrelevant TABs. If clinical suspicion of GCA is high, a TAB can be considered clinically irrelevant.

Propylthiouracil-induced lupus-like or vasculitis syndrome

A 27 year old female with Graves’ disease presented with fever, exertional dyspnea and polyarthralgia. Erythema nodosum had occured three months earlier. The patient declared irregular use of propylthiouracil (PTU) for the last 8 months. Neutropenia and microscopic hematuria developed in the second week of admission. Chest X-ray showed inhomogenous pulmonary opacities, left pleural effusion and cardiomegaly. Computed tomography (CT) revealed multiple subpleural nodules, left pleural effusion, pericardial effusion, enlarged mediastinal and axillary lymph nodes. Bronchoalveolar lavage (BAL) cytology demonstrated hemosiderin laden macrophages. Histopathologic examination of the transbronchial biopsy specimen revealed a nonspecific inflammation. Serum was positive for ANA, P-ANCA, MPO-ANCA, PR3-ANCA and negative for anti-ds-DNA, C-ANCA, C3, C4 and anti-histone antibody. All symptoms resolved in two months after PTU withdrawal and starting steroid treatment. The same clinical manifestations recurred when the patient used PTU erronously one month after discharge.This is a case of PTU induced-autoimmune disease in whom the accurate distinction between drug-induced-lupus (DIL) and vasculitis was not possible due to the significant overlap of clinical and laboratory findings causing a significant diagnostic challenge for the chest physician.

Propylthiouracil-induced lupus-like or vasculitis syndrome

A 27 year old female with Graves’ disease presented with fever, exertional dyspnea and polyarthralgia. Erythema nodosum had occured three months earlier. The patient declared irregular use of propylthiouracil (PTU) for the last 8 months. Neutropenia and microscopic hematuria developed in the second week of admission. Chest X-ray showed inhomogenous pulmonary opacities, left pleural effusion and cardiomegaly. Computed tomography (CT) revealed multiple subpleural nodules, left pleural effusion, pericardial effusion, enlarged mediastinal and axillary lymph nodes. Bronchoalveolar lavage (BAL) cytology demonstrated hemosiderin laden macrophages. Histopathologic examination of the transbronchial biopsy specimen revealed a nonspecific inflammation. Serum was positive for ANA, P-ANCA, MPO-ANCA, PR3-ANCA and negative for anti-ds-DNA, C-ANCA, C3, C4 and antihistone antibody. All symptoms resolved in two months after PTU withdrawal and starting steroid treatment. The same clinical manifestations recurred when the patient used PTU erronously one month after discharge. This is a case of PTU induced-autoimmune disease in whom the accurate distinction between drug-induced-lupus (DIL) and vasculitis was not possible due to the significant overlap of clinical and laboratory findings causing a significant diagnostic challenge for the chest physician.

Autoimmune Pancreatitis Presenting as a Focal Pancreatic Mass Mimicking Pancreatic Cancer: A Case Report and Review of the Literature

A 68-year-old female presented with a 2-week history of jaundice, generalized itching and weight loss in 2006. On physical examination, she was deeply jaundiced; however, other clinical examinations were completely unremarkable. Liver function tests (LFTs) were deranged: serum alkaline phosphatase 407 U/L (35 - 104), serum aspartate transaminase 57 U/L (0 - 31), serum alanine transaminase 184 U/L (0 - 37), and serum total bilirubin 4.3 mg/dL (0 - 1). Abdominal CT revealed a 4.8 cm × 3.5 cm mass in the pancreatic head. ERCP revealed a high-grade stricture in the distal CBD. An internal biliary stent was placed in to the CBD stricture. Patient’s symptoms relieved after biliary stenting. Serum CA 19-9 was elevated at 1,568 U/mL (0 - 37). Due to high suspicion for pancreatic cancer, patient underwent pylorus-sparing pancreaticoduodenectomy. Histopathology was benign and showed perilobular lymphoplasmacytic infiltrate, periductal fibrosis and phlebitis that was consistent with autoimmune pancreatitis. Patient was started on oral prednisone that was eventually replaced with azathioprine. Serum IgG4 was 53 mg/dL (1 - 291) after starting steroid treatment. J Med Cases. 2013;4(1):29-33 doi: https://doi.org/10.4021/jmc891w

Elevated Absolute Lymphocyte Counts Following Acute Graft Vs. Host Disease Treatment Is Associated with Therapeutic Responses and Lower Non-Relapse Mortality Following Umbilical Cord Blood Transplant,

Abstract 4085Acute graft vs. host diseases (aGVHD) is a life threatening complication of allogeneic hematopoietic cell transplantation (allo-HCT). In a large series of patients undergoing allo-HCT (n=863) we recently demonstrated that following the initiation of corticosteroids, a clinical response at day 28 was associated with an increased likelihood of overall treatment response and a reduction in treatment related mortality (TRM) (MacMillan, Blood, 2010). We have also shown that patients who have rapid lymphocyte recovery following umbilical cord blood transplantation (UCBT) have improved outcomes (Burke, BBMT, 2010). To date, no study has addressed the influence of absolute lymphocyte count (ALC) on aGVHD treatment responses. We hypothesized that high ALC might predict favorable responses to aGVHD therapy. To test this hypothesis we reviewed the ALC at the time of aGVHD diagnosis and weekly following the initiation of corticosteroid therapy (48 mg/m2 × 14 days followed by a 10% taper over 8 weeks). ALCs were collected on patients who had clinical laboratory data available in the electronic medical record and who had available data on clinical response to corticosteroid therapy. There were 211 patients transplanted at our center from 2001–2007 who fit the above criteria. Median age was 42 years (range 0.2–69 yrs). Thirty nine patients (19%) were <18 years of age and 135 (64%) were >35 years old. The majority of patients underwent UCBT (n=134, 64%) and most others received a sibling PBSC transplant (n=71, 34%). Myeloablative conditioning was used in 55% of patients. GVHD prophylaxis was mainly with CSA/MMF (n=142, 67%) or MTX/CSA (n=42, 22%). Median time to aGVHD onset was 37 days (11–92). Patterns of aGVHD included skin only (n=91, 43%), gut only (n=36, 17%) or multi-organ involvement (n=82, 39%). At the time of GVHD diagnosis, ALC was not predictive of response to therapy. Likewise, there was no association with the ALC at D7 (after the start of steroid treatment) and therapeutic response. The D14 post-treatment ALC showed an association with clinical response at D28 (a time point previously associated with long-term responses and NRM). Patients with an ALC of 0–0.14, 0.15–0.34 and >0.35 at D+14 after corticosteroid therapy had a 40%, 54% and 68% chance of a clinical response at D28 (p>0.01). This translated into a reduction in non-relapse mortality for patients with higher ALC (0–0.14 vs. >0.15; 33% [19–47%] vs 23% [17–29%], p=0.04). Subgroup analysis showed that these observations were mainly driven by the myeloablatve UCB group. Treating ALC as a continuous variable and using a repeated measures approach, we observed that for every unit increase in ALC between the day of diagnosis and D14, there was a 2.26 higher increased likelihood of having a complete clinical response at D28 (p>0.001). Similar results were seen for NRM (OR=0.89 [0.81–9.8], p=0.01). In multivariate analysis, repeated measures of ALC (from D0-14) remained significant for both D28 clinical response (p=0.001) and NRM (p=0.05). Patients with steroid refractory aGVHD (n=17) were treated with ATG. Because steroid refractory aGVHD has poor outcomes and because ATG can negatively impact ALC, we removed these patients from the analysis to determine the impact of ALC. In this subgroup analysis, ALC at D14 after aGVHD treatment remained associated with D28 clinical response in multivariate analysis (OR=3.01, 95%CI[1.24–7.35], p=0.02). Using repeated analysis, the change in ALC from aGVHD treatment day D0 to 14 was associated with D28 treatment response (OR=4.42 [1.88–10.37], p<0.001). Collectively, these results show that absolute ALC at D14 and increases in ALC from the day of treatment to day 14were associated with D28 clinical response and NRM. ALC maybe a simple and cost effective method to monitor response to aGVHD therapy. Disclosures:No relevant conflicts of interest to declare.

Steroid treatment alters adhesion molecule and chemokine expression in experimental acute graft-vs.-host disease of the intestinal tract

Acute graft-vs.-host disease (aGVHD) is a major complication after allogeneic bone marrow transplantation (allo-BMT) that is characterized by high morbidity and mortality. Systemic treatment with steroids has been the mainstay of first-line therapy of aGVHD, although controlled experimental data in this context are limited. Using a haploidentical murine BMT model, steroid effects on hepatic and intestinal inflammation during aGVHD have been investigated. Lethally irradiated B6D2F1 mice received bone marrow cells and splenocytes from either syngeneic (B6D2F1) or allogeneic (C57BL/6) donors. Intraperitoneal administration of prednisolone (2 mg/kg body weight every day) early after onset of GVHD from day +10 until day +42 resulted in reduced clinical GVHD severity and improved survival of allogeneic recipients. Although the liver was barely affected by prednisolone treatment, aGVHD-related histopathologic injury of the gastrointestinal tract was strongly reduced in association with diminished expression of interferon-γ, tumor necrosis factor, CXCL 9-11, CCL2-3, mucosal addressin cell adhesion molecule-1, and intercellular adhesion molecule-1. Prednisolone-induced reduction of adhesion molecule expression in the gut manifested earlier than seen for cytokines or chemokines. Interestingly, when starting steroid treatment on day +28, the course of GVHD was unchanged and no major differences in cyto- or chemokine expression in gastrointestinal tract or liver on day +42 were seen. When started early after GVHD onset, prednisolone-related beneficial effects can affect aGVHD target organs differently, involving divergent regulation of inflammation and leukocyte migration. Specifically, a change in adhesion properties between leukocytes and endothelial cells in the gastrointestinal tract may be one of the initial steps in a cascade of steroid-related aGVHD-attenuating events.

Standard steroid treatment for autoimmune pancreatitis

Objective:To establish an appropriate steroid treatment regimen for autoimmune pancreatitis (AIP).Methods:A retrospective survey of AIP treatment was conducted in 17 centres in Japan. The main outcome measures were rate of...

Glucocorticoids increase CD4+CD25highcell percentage and Foxp3 expression in patients with multiple sclerosis

To determine whether percentages of CD4(+)CD25(high) T cells (a group of regulatory T cells, Treg) differ in patients with multiple sclerosis (MS) in relapse vs remission after glucocorticoid treatment and whether treatment for relapses changes Treg population and the expression of Foxp3, a key Treg-associated molecule. Peripheral blood mononuclear cells (PBMC) were obtained from 20 patients with MS during relapse, just before and 2 days after starting steroid treatment (i.v. methylprednisolone 1 g/day for 3 days) and then 6 weeks after treatment. CD4(+)CD25(hi) cells were analysed by using flow cytometry. Cytokines were measured by using an ELISA and Foxp3, CD3 and CD25 expression by using quantitative real-time PCR. The percentage of CD4(+)CD25(hi) cells, plasma IL-10 and Foxp3/CD3 ratio increased 48 h after methylprednisolone initiation and returned to baseline values by 6 weeks post-treatment. Results suggest that glucocorticoids increase Treg cell functional molecules and percentages. This may be a mechanism whereby steroids expedite recovery from MS relapses.

Evidence of a sub-clinical lymphoproliferative disease in patients with ‘idiopathic‘ autoimmune hemolytic anemia

7074 Background: Idiopathic autoimmune hemolytic anemia (AHA), which accounts for about half of cases, is diagnosed in the absence of a concomitant disease. A lymphoproliferative disease is frequently associated or can follow AHA. The aim of this study was to better define the clinical features of 50 cases defined as idiopathic AHA. Methods: Before starting steroid treatment, the following examinations were performed: immunohematologic study; total body CT scan or a chest XR with abdomen ultrasound; HBV and HCV serology; autoantibody screening (anti-nucleous; anti-cardiolipin; anti-gastric parietal cell; anti-beta 2-glycoprotein-I); peripheral blood (PB) immunophenotype. Results: The median age of the 50 patients was 66 years (range: 20–84), 32 patients were females. The immunohematologic study revealed an anti-erythrocyte auto-antibody in all cases (IgG: 27; IgM:15; IgG + IgM: 7; IgA:1). An idiopathic AHA was confirmed in 26 cases (52%), while in 24 (48%) a concurrent disease was diagnosed (tumor: 4; HCV hepatitis: 2; autoimmune disease: 2). In 16 cases (32 %), all with a normal lymphocyte count, the PB immunophenotype revealed the presence of a small B-lymphocyte clone with a CD5+/CD19+, CD23+ phenotype in 3 cases and a CD5+/CD19+, CD23- or CD5-/CD19+, CD23- phenotype in 13. The median number of clonal B lymphocytes in the PB was 0.5 x 109/L (range: 0.03–1.9 x 109/L). Clonality was confirmed by PCR. The same clonal population was detected in the bone marrow aspirate. No other signs ascribable to a lymphoproliferative disease were detected, except for a patient with an enlarged abdominal lymph node. Conclusions: Our findings indicate that about one third of newly diagnosed patients with otherwise defined “idiopathic” AHA show a underlined sub clinical B-lymphocyte clone which may play a role both in the pathogenesis of the autoimmune disorder and in the subsequent occurrence of a lymphoproliferative disease. The detection of such clones should be considered in the diagnostic work-out of patients with AHA. No significant financial relationships to disclose.

Spontaneous Regression of Steroid-related Osteonecrosis of the Knee

It is unknown whether lesions of steroid-related osteonecrosis of the knee increase or decrease in size during the course of the disease after diagnosis. We sought to determine whether steroid-related osteonecrosis of the knee would have spontaneous changes in size, and if so, the factors affecting the change. We performed baseline and followup (minimum of 1 year) magnetic resonance imaging scans on 30 knees of 17 patients. We then used image registration techniques to match two sets of images. Lesion size change was evaluated on all contiguous pairs of matched magnetic resonance images. Fourteen Stage 1 (preradiographic stage) knees in seven patients showed spontaneous incomplete regression without subsequent collapse. These patients had early steroid-related lesions detected within 3 years after starting steroid treatment and all showed bilateral and multifocal involvement; lesion regression occurred regardless of location. The initial size and location of the lesions and discontinuing steroid administration did not seem to affect regression. Regression can occur in some patients with early steroid-related osteonecrosis of the knee, and the time between initiation of steroid treatment and its diagnosis might be the most significant predictive factor.