Clinical, Endocrine and Imaging Characteristics of Patients with Primary Hypophysitis

2018 ◽  
Vol 50 (04) ◽  
pp. 296-302 ◽  
Author(s):  
Anna Angelousi ◽  
Carolina Cohen ◽  
Soledad Sosa ◽  
Karina Danilowicz ◽  
Lina Papanastasiou ◽  
...  
Keyword(s):  
Natural History ◽  
Immunosuppressive Agents ◽  
Hormonal Treatment ◽  
Response To Treatment ◽  
High Dose ◽  
Partial Recovery ◽  
Gonadal Dysfunction ◽  
Imaging Characteristics ◽  
Primary Hypophysitis

AbstractPrimary hypophysitis (PH) is a rare disease with a poorly-defined natural history. Our aim was to characterise patients with PH at presentation and during prolonged follow-up. Observational retrospective study of 22 patients was conducted from 3 centres. In 14 patients, PH was confirmed histologically and in the remaining 8 clinically, after excluding secondary causes of hypophysitis. All patients had hormonal and imaging investigations before any treatment. Median follow up was 48 months (25–75%: 3–60). There was a female predominance with a female/male ratio: 3.4:1. Eight out of 22 patients had another autoimmune disease. Headaches and gonadal dysfunction were the most common symptoms. Five patients presented with panhypopituitarism; 17 patients had anterior pituitary deficiency, and 7 had diabetes insipidus. At presentation, 9 patients were treated surgically, 5 received replacement hormonal treatment, and 8 high-dose glucocorticoids from whom 5 in association with other immunosuppressive agents. Six patients showed complete recovery of pituitary hormonal deficiencies while 6 showed a partial recovery during a 5-year follow-up period. No difference was found between patients treated with surgery and those treated medically. The overall relapse rate was 18%. PH can be manifested with a broad spectrum of clinical and hormonal disturbances. Long-term follow-up is required to define the natural history of the disease and response to treatment, since pituitary hormonal recovery or relapse may appear many years after initial diagnosis. We suggest that surgery and immunosuppressive therapy be reserved for exceptional cases.

A Follow-up Study of Psychiatric Disturbance in a Cape Coloured Community

1973 ◽  
Vol 123 (574) ◽  
pp. 279-283 ◽  
Author(s):  
L. S. Gillis ◽  
G. L. Stone
Keyword(s):  
Mental Disorders ◽  
Natural History ◽  
Psychiatric Disorders ◽  
Longitudinal Studies ◽  
Response To Treatment ◽  
Psychiatric Disturbance ◽  
Follow Up Study ◽  
History Of

Longitudinal studies of psychiatric disturbance in communities are important in order to determine the natural history of mental disorders. Most studies have focused on the prevalence of known psychiatric disorders and response to treatment, but only a few on the follow up of a population not previously recognized as psychiatrically ill (Beiser (1), Hagnell (5), Helgason (6), Myers and Bean (10)). The present study is an attempt to do this, and also to follow up untreated disorder within a community.

The natural history of pineal cysts in children and young adults

2010 ◽  
Vol 5 (2) ◽  
pp. 162-166 ◽  
Author(s):  
Wajd N. Al-Holou ◽  
Cormac O. Maher ◽  
Karin M. Muraszko ◽  
Hugh J. L. Garton
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Initial Study ◽  
Older Children ◽  
Imaging Evaluation ◽  
Imaging Appearance ◽  
Imaging Characteristics ◽  
History Of ◽  
The Mean

Object The authors reviewed their experience with pediatric pineal cysts to define the natural history and clinical relevance of this common intracranial finding. Methods The authors identified all patients with pineal cysts who had been clinically evaluated at their institution over an 11.5-year interval and were < 25 years of age at the time of diagnosis. All inclusion criteria were met in 106 patients, and included repeated MR imaging as well as repeated clinical evaluation over at least a 6-month interval. Results The mean age at diagnosis was 11.7 ± 7.2 years. Forty-two patients were male and 64 were female. On follow-up MR imaging evaluation at a mean interval of 3.0 years from the initial study, 98 pineal cysts had no increase in size and no change in imaging appearance. Six pineal cysts increased in size and 2 others had a change in imaging characteristics without associated growth. Younger age was associated with cyst change or growth on follow-up imaging (p = 0.02). The mean age of patients with cysts that changed or grew was 5.5 years, and the mean age of patients with stable pineal cysts was 12.2 years. Initial cyst size and appearance on MR imaging were not significant predictors of growth or change in imaging appearance at follow-up. Similarly, the patient's sex was not a significant predictor of growth or change in imaging characteristics. Conclusions Follow-up imaging and neurosurgical evaluation may be considered optional in older children with pineal cysts.

Has the Incidence of Extramedullary Disease Changed with the New Therapeutic Approaches? Analysis of a Cohort of 965 Multiple Myeloma (MM) Patients (pts).

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4749-4749
Author(s):  
Marzia Varettoni ◽  
Alessandro Corso ◽  
Gianmatteo Pica ◽  
Patrizia Zappasodi ◽  
Silvia Mangiacavalli ◽  
...  
Keyword(s):  
Overall Survival ◽  
Median Time ◽  
Response To Therapy ◽  
Response To Treatment ◽  
High Dose ◽  
Solitary Plasmacytoma ◽  
Therapeutic Approaches ◽  
New Therapeutic Approaches ◽  
Over Time

Abstract Extramedullary myeloma (EMM) at diagnosis or during the course of the disease is rare and often anecdotal. We reviewed the records of 965 consecutive MM pts diagnosed and followed from 1969 to July 2007 in order to evaluate: the overall incidence of EMMs and the changes over time clinical presentation, response to treatment and outcome of EMM pts divided into two subgroups according to the time of appearance, at diagnosis or during the course of the disease. We considered three periods: 1969–1989 (conventional chemotherapy, CCT); 1990–1999 (introduction of high-dose therapy, HDT); 2000–2007, (era of novel agents). The overall incidence of EMM was of 13% (129/965 pts), 87/42 M/F, median age 58 (31–80) years. A prior MGUS was present in 24 pts and a solitary plasmacytoma in 10 (8%). Characteristics at the time of EMM were: 77 pts IgG (60%), 23 IgA (18%), 2 IgM (2%), 16 light chain (12%), 11 not secretory (8%); 29 pts were in stage I, 14 in stage II, 86 in stage III; 30/129 pts (23%) were asymptomatic. More frequently involved sites were: paravertebral (40%), rib cage (32%), pelvis (10%). Multiple localizations were present in 27 pts (21%). A plasmacytic leukemia was observed during the follow-up in 9 pts (7%). The overall median follow-up was 24.4 (2.5–148) months. Seventy-three pts presented EMM at the time of diagnosis with different incidences in the 3 periods: 1969–1989 4.5%, 1990–99 4.3%, 2000–07 12.7%. These pts were treated with HDT in 43 cases (59%) and CCT in 30 (41%). Radiotherapy (RT) was associated in 38 pts (52%). A partial response (PR) was achieved in 49 pts (67%). Progression or relapse were observed in 46 pts (63%) and the median time to progression (TTP) and overall survival (OS) in this subgroup of pts were 17.3 and 21.5 months respectively. The other 56 pts showed an EMM during the course of the disease after a median time of 20 (2–144) months from diagnosis. EMM incidence varied as follows: 1969–1989 2.7%, 1990–99 7.2%, 2000–07 7.4%. Median number of previous lines of therapy was 1 (range: 1–7), including HDT in 22 pts (39%), thalidomide or lenalidomide in 18 (32%), bortezomib in 5 (9%). The median time from HDT to EMM was 17 (2–125) months. Treatment of these pts consisted of CCT in 36 cases (64%), thalidomide in 3 (5%) and bortezomib in 8 (14%). RT was given in 29 cases (52%). Response rate in this subgroup was low, only 30% of pts obtained a PR. The median TTP and OS from the time of appearance of EMM were 4.7 and 6.5 months respectively and the overall survival from the diagnosis was 29.9 months. The two groups of EMM pts were also compared for all the clinical characteristics, response to therapy and outcome. EMM pts at diagnosis showed higher levels of monoclonal component and haemoglobin, and lower bone marrow plasmacytosis with respect to the others. OS from diagnosis was similar in the two groups. In conclusion, our study shows an increased incidence of EMM over time. The more recent increase of EMM at diagnosis might be due to the wider use of more sensitive imaging techniques as CT scan and magnetic resonance, while during the course of the disease after 1990 could be related to the longer survival of pts thanks to the new therapeutic approaches. Anyway, the presence of EMM whether at diagnosis or at progression seems to negatively affect the outcome of pts since the OS is shorter than MM pts without EMM.

Prevalence and natural history of arachnoid cysts in children

2010 ◽  
Vol 5 (6) ◽  
pp. 578-585 ◽  
Author(s):  
Wajd N. Al-Holou ◽  
Andrew Y. Yew ◽  
Zackary E. Boomsaad ◽  
Hugh J. L. Garton ◽  
Karin M. Muraszko ◽  
...  
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Incidental Finding ◽  
Arachnoid Cysts ◽  
Consecutive Series ◽  
Imaging Characteristics ◽  
History Analysis ◽  
History Of ◽  
Over Time

Object Arachnoid cysts are a frequent finding on intracranial imaging in children. The prevalence and natural history of these cysts are not well defined. The authors studied a large consecutive series of children undergoing MR imaging to better define both the MR imaging–demonstrated prevalence and behavior of these lesions over time. Methods The authors reviewed a consecutive series of 11,738 patients who were 18 years of age or younger and had undergone brain MR imaging at a single institution during an 11-year period. In the patients in whom intracranial arachnoid cysts were identified, clinical and demographic information was recorded and imaging characteristics, such as cyst size and location, were evaluated. Prevalence data were analyzed using univariate and multivariate logistic regression, linear regression, and ANOVA. All patients with sufficient data (repeat MR imaging studies as well as repeated clinical evaluation over at least 5 months) for a natural history analysis were identified. This group was assessed for any change in symptoms or imaging appearance during the follow-up interval. Results Three hundred nine arachnoid cysts (2.6% prevalence rate) were identified. There was an increased prevalence of arachnoid cysts in males (p < 0.000001). One hundred eleven patients met all criteria for inclusion in the natural history analysis. After a mean follow-up of 3.5 years, 11 arachnoid cysts increased in size, 13 decreased, and 87 remained stable. A younger age at presentation was significantly associated with cyst enlargement (p = 0.001) and the need for surgery (p = 0.05). No patient older than 4 years of age at the time of initial diagnosis had cyst enlargement, demonstrated new symptoms, or underwent surgical treatment. Conclusions Arachnoid cysts are a common incidental finding on intracranial imaging in pediatric patients. An older age at the time of presentation is associated with a lack of clinical or imaging changes over time.

ICHD-II Diagnostic Criteria for Tolosa—Hunt Syndrome in Idiopathic Inflammatory Syndromes of the Orbit and/or the Cavernous Sinus

Cephalalgia ◽  
2008 ◽  
Vol 28 (6) ◽  
pp. 577-584 ◽  
Author(s):  
S Colnaghi ◽  
M Versino ◽  
E Marchioni ◽  
A Pichiecchio ◽  
S Bastianello ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Response To Treatment ◽  
High Dose ◽  
Inclusion Criteria ◽  
Resonance Imaging ◽  
Hunt Syndrome ◽  
Magnetic Resonance Imaging Mri

A bibliographical search was conducted for papers published between 1999 and 2007 to verify the validity of International Classification of Headache Disorders (ICHD)-II criteria for the Tolosa-Hunt syndrome (THS) in terms of (i) the role of magnetic resonance imaging (MRI); (ii) which steroid treatment should be considered as adequate; and (iii) the response to treatment. Of 536 articles, 48, reporting on 62 patients, met the inclusion criteria. MRI was positive in 92.1% of the cases and it normalized after clinical resolution. There was no evidence of which steroid schedule should be considered as adequate; high-dose steroids are likely to be more effective both to induce resolution and to avoid recurrences. Pain subsided within the time limit required by the ICHD-II criteria, but signs did not. We conclude that THS diagnostic criteria can be improved on the basis of currently available data. MRI should play a pivotal role both to diagnose and to follow-up THS.

scholarly journals Real-World Data with the Use of Caplacizumab in the Treatment of Acquired Thrombotic Thrombocytopenic Purpura (aTTP)

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 14-16
Author(s):  
M Moraima Jimenez ◽  
Sabela Bobillo ◽  
Ana Pérez ◽  
Pamela Arenas ◽  
Alba Cabirta ◽  
...  
Keyword(s):  
Real World ◽  
Clinical Characteristics ◽  
Randomized Clinical Trials ◽  
Immunosuppressive Agents ◽  
Response To Treatment ◽  
Lower Percentage ◽  
Neurological Involvement ◽  
Control Group ◽  
Real World Data

INTRODUCTION For more than two decades, the treatment of aTTPconsisted of therapeutic plasma exchange (TPE) and immunosuppressive agents. The addition of caplacizumab, a nano-antibody that binds to the A1 domain of the von Willebrand factor, inhibiting platelet aggregation, has been shown to reduce the time to resolution of thrombocytopenia, the rate of recurrence and the aTTP-related death. Real-world evidence of the effectiveness of caplacizumab is limited yet. The objective of our study was to assess the results of the introduction of caplacizumab in our internal protocol and to compare those results with the patients treated before the drug was available. METHODS A single-center retrospective observational study that evaluates the clinical characteristics and response to treatment of 18 consecutively diagnosed aTTP patients between May/14 to May/20. All patients received initial treatment with TPE and prednisone (PDN) 1 mg/Kg; the control group did not receive any other initial therapy, whereas nine patients received caplacizumab in addition to PEX and PDN once ADAMTS-13 deficiency was confirmed. Complete response (CR) was defined as the second of two consecutive days with platelets ≥150x109/L, refractoriness as the lack of platelet increase despite optimal therapy after 7 days, exacerbation as the decrease in platelet count during the first 30 days of discontinuation of TPE, and relapse as a new episode of aTTP beyond 30 days after the last TPE. All results are given as median (interquartile range). Statistical analysis was conducted using STATA/IC software. RESULTS The clinical characteristics at diagnosis of patients treated with or without caplacizumab were similar, except for a lower percentage of males and lower neurological involvement in the caplacizumab group (Table 1). Caplacizumab was started at a median of 3 days after diagnosis following ADAMTS-13 deficiency determination, and was administered during a median of 39 days (IQR 33-39). Adverse events related to caplacizumab were mild: 1 patient presented mild metrorrhagia, 1 developed pain and erythema at the puncture area and 1 suffered an urticarial dermatitis, the last case leading to the suspension of the drug since levels of ADAMTS-13 were recovered. The caplacizumab group achieved CR after a median of 4 days (IQR 3-4) vs. 6 days (IQR 5-14) in the control group (p = 0.016). Likewise, the number of TPE was lower with caplacizumab (Figure 1), with a median of 10 TPE (IQR 9-11) vs. 19 (IQR 16-23) (p = 0.001). Hospitalization time was also shorter in the caplacizumab group with a median of 12 days (IQR 12-14) vs. 26 (IQR 20-27) (p = 0.002). Finally the time of hospitalization into the intensive care unit was shorter in the caplacizumab group with a median of 3 days (IQR 2-4) vs. 4 (IQR 3-13) (p=0.1). In the caplacizumab group (median follow-up of 6.8 months), there were no refractory cases. There was 1 exacerbation before initiation of caplacizumab and 1 relapse. Both cases were treated with rituximab. In contrast, in the control group (median follow-up of 51.8 months), we observed 4 refractory cases (1 aTTP-related death), 3 exacerbations and 1 relapse; rituximab was necessary in 8 patients and a 3rd line with vincristine was administered in 4 cases. CONCLUSIONS The observed benefits of caplacizumab in our series are in line with the ones identified in randomized clinical trials. Caplacizumab can be used in combination with other therapies to attain a faster response and reduce aTTP-related complications. Disclosures Bosch: Hoffmann-La Roche: Research Funding.

scholarly journals IgM Mesangial Deposition Predicts Relapses of Adult-Onset Minimal Change Disease

2020 ◽  
Author(s):  
Cheng-Wen Yang ◽  
Fan-Yu Chen ◽  
Fu-Pang Chang ◽  
Yang Ho ◽  
An-Hang Yang ◽  
...  
Keyword(s):  
Minimal Change Disease ◽  
Immunosuppressive Agents ◽  
Immunosuppressive Treatment ◽  
Minimal Change ◽  
Immunoglobulin M ◽  
Response To Treatment ◽  
Adult Onset ◽  
Significant Difference ◽  
The Impact

Abstract Background: Immunoglobulin M (IgM) mesangial deposition in pediatric minimal change disease (MCD) has been reported to be associated with steroid dependence and poor renal outcomes. However, the evidence regarding the impact of IgM mesangial deposition on the treatment responses or outcomes in adult-onset MCD is lacking.Methods: In this retrospective cohort study, 37 adult patients with MCD received kidney biopsy from January 2010 to May 2020. According to IgM mesangial deposition by immunofluorescence microscopy, the patients were divided into two groups (12 patients with positive IgM deposition; 25 patients with negative IgM deposition). We analyzed the clinical features, the dosage of immunosuppressive agents, and the response to treatment for two years between the two groups.Results: Regarding the clinical symptoms, the dosage of immunosuppressive treatment, and the time to remission, there was no statistically significant difference between the two groups. Compared to the negative IgM group, the frequency of relapses was significantly higher in the positive IgM group within the two-year follow-up period (the negative IgM group 0.25 episodes/year; the positive IgM group 0.75 episodes/year, p = 0.029). Furthermore, multivariate linear regression revealed that the positivity of IgM mesangial deposition is independently associated with the frequency of relapses (regression coefficient B 0.450, 95% CI 0.116-0.784, p = 0.010).Conclusions: Our findings indicated that adult-onset MCD patients with IgM mesangial deposition have a high risk of relapses. Therefore, prolonged and combined immunosuppressive therapy with close follow-up may be considered in MCD adults with IgM mesangial deposition.

scholarly journals Rajaie Cardiomyopathy and Myocarditis Registry: Protocol for an Observational Study

2021 ◽  
Author(s):  
Majid Maleki ◽  
Freidoun Noohi ◽  
Parham Sadeghipour ◽  
Mohammad Mehdi Peighambari ◽  
Ahmad Amin ◽  
...  
Keyword(s):  
Natural History ◽  
Clinical Signs ◽  
Response To Treatment ◽  
Cardiac Resynchronization ◽  
Therapeutic Approaches ◽  
Long Term Outcomes ◽  
Prediction Of Mortality ◽  
Number Of Patients

Most of the information on the natural history and management of cardiomyopathies and myocarditis in Iran has been obtained from cohort studies in a small number of patients. The prevalence of patients with cardiomyopathies referred to Rajaei Cardiovascular medical and research centers from all over the country is remarkable. Rajaie Cardiomyopathy and myocarditis Registry (RCMR) study is an observational registry of patients with four subtype of cardiomyopathy include: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM) as well as myocarditis designed to determine clinical characteristics, natural history, current therapeutic approaches, response to treatment and long-term outcomes of patients with cardiomyopathy and myocarditis. Prediction of mortality and response to different treatments in these patients using artificial intelligence is another aim of this Registry. COVID 19 Myocarditis and its sequence as cardiomyopathy seem a new challenge in forthcoming years. At the baseline visit, past medical history, clinical signs/symptoms, risk factors, physical examination and family history of cardiomyopathy, current standards for diagnostic workup and clinical follow-up, and relevant electrocardiogram echocardiography, cardiac magnetic resonance, Holter monitoring, or biomarker analyses will be checked. The outcome and results of various therapeutic approaches currently employed for patients, including implantable cardioverter defibrillator, cardiac resynchronization therapy, septal myomectomy, ablation, cardiac transplantation, and medications, will be assessed. Long-term outcomes, including the benefits and complications of therapeutic interventions, will be collected. A follow-up visit will be scheduled after 12 months for all patients, and survival status, hospitalizations, co-morbidities, medications will be assessed.

The natural history of familial cavernous malformations: results of an ongoing study

1994 ◽  
Vol 80 (3) ◽  
pp. 422-432 ◽  
Author(s):  
Joseph M. Zabramski ◽  
Thomas M. Wascher ◽  
Robert F. Spetzler ◽  
Blake Johnson ◽  
John Golfinos ◽  
...  
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Ongoing Study ◽  
Mr Images ◽  
Cavernous Malformations ◽  
Content Type ◽  
Imaging Characteristics ◽  
Familial Form ◽  
Fine Print

✓ Cavernous malformations are congenital abnormalities of the cerebral vessels that affect 0.5% to 0.7% of the population. They occur in two forms: a sporadic form characterized by isolated lesions, and a familial form characterized by multiple lesions with an autosomal dominant mode of inheritance. The management of patients with cavernous malformations, particularly those with the familial form of the disease, remains a challenge because little is known regarding the natural history. The authors report the results of an ongoing study in which six families afflicted by familial cavernous malformations have been prospectively followed with serial interviews, physical examinations, and magnetic resonance (MR) imaging at 6- to 12-month intervals. A total of 59 members of these six families were screened for protocol enrollment; 31 (53%) had MR evidence of familial cavernous malformations. Nineteen (61%) of these 31 patients were symptomatic, with seizures in 12 (39%), recurrent headaches in 16 (52%), focal sensory/motor deficits in three (10%), and visual field deficits in two (6%). Twenty-one of these 31 patients underwent at least two serial clinical and MR imaging examinations. A total of 128 individual cavernous malformations (mean 6.5 ± 3.8 lesions/patient) were identified and followed radiographically. During a mean follow-up period of 2.2 years (range 1 to 5.5 years), serial MR images demonstrated 17 new lesions in six (29%) of the 21 patients; 13 lesions (10%) showed changes in signal characteristics, and five lesions (3.9%) changed significantly in size. The incidence of symptomatic hemorrhage was 1.1% per lesion per year. The results of this study demonstrate that the familial form of cavernous malformations is a dynamic disease; serial MR images revealed changes in the number, size, and imaging characteristics of lesions consistent with acute or resolving hemorrhage. It is believed that the de novo development of new lesions in this disease has not been previously reported. These findings suggest that patients with familial cavernous malformations require careful follow-up monitoring, and that significant changes in neurological symptoms warrant repeat MR imaging. Surgery should be considered only for lesions that produce repetitive or progressive symptoms. Prophylactic resection of asymptomatic lesions does not appear to be indicated.

Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1961-1967
Author(s):  
Wanlu Ma ◽  
Wei Bai ◽  
Xueyan Wu ◽  
Jiuliang Zhao ◽  
Mengtao Li ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
Immunosuppressive Agents ◽  
High Dose ◽  
Systemic Lupus ◽  
College Hospital ◽  
Usual Practice ◽  
Platelet Counts

Objectives Thrombotic thrombocytopenia purpura (TTP) associated with systemic lupus erythematous (SLE) (i.e., SLE-TTP) is a rare life-threatening disease often requiring intensive immunosuppressive agents, in addition to high-dose corticosteroids and plasma exchange (PEX). The optimal therapy of rituximab is unclear, but 375 mg/m2 weekly for 4 weeks is the usual practice, adopted from regimens for non-Hodgkin’s lymphoma. We reported two cases of refractory SLE-TTP that showed good efficacy and prognosis with combination of methylprednisolone (MP) pulse, plasma exchange and low-dose rituximab (100 mg weekly for 4 weeks) treatment. Methods Clinical data and treatment outcomes were reviewed of two patients diagnosed with refractory SLE-TTP at Peking Union Medical College Hospital between July 2017 and July 2018. Results Both patients had SLE and presented with microangiopathic anemia and thrombocytopenia. Laboratory assays revealed high anti-nuclear antibody titers, reduced complement 3 and 4 levels, proteinuria, significantly elevated lactate dehydrogenase, schistocytes on peripheral blood smear, low ADAMTS13 activity, and the presence of ADAMTS13 inhibitor. In both patients, platelet counts remained below 50 × 109/L after MP pulse and 6 PEXs, confirming the diagnosis of refractory SLE-TTP. Low-dose rituximab (100 mg weekly for 4 weeks) was administered in both cases, resulting in normalization of platelet counts and significant reductions in B-lymphocyte counts. No TTP relapse or SLE flare occurred during 24 months of follow-up. Conclusions Our cases confirmed the efficacy and good follow-up outcomes of low-dose rituximab treatment (100 mg weekly for 4 weeks) for refractory SLE-TTP.

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