Persistent Clinical Remission Research Articles

The application value of MRI in the diagnosis of subclinical inflammation in patients with rheumatoid arthritis in remission

BackgroundTo explore the value of MRI in the diagnosis of subclinical inflammation in patients with early rheumatoid arthritis (RA) in remission and to predict the radiographic progression.MethodsA total of 76 of 156 patients with early RA in remission at 1 year and with available magnetic resonance imaging (MRI) data at baseline and at 12 months were included. Complete clinical and laboratory evaluations were conducted for the patients. MRI images were assessed according to the Rheumatoid Arthritis Magnetic Resonance Imaging Scoring (RAMRIS) system. Progression of bone erosions was defined as an increase of 1 or more units in annual RAMRIS score for erosions compared to baseline.ResultsAt 1 year, the majority of patients with RA in sustained remission showed some inflammatory activity on MRI (43.4% synovitis, 39.5% bone marrow edema (BME), and 9.2% tenosynovitis), and 25 of the 76 patients (32.9%) showed MRI progression of bone erosions. A significant difference was observed in MRI BME and bone erosion at 1 year, with higher mean score in patients with progression compared to non-progression of erosions (BME, 4.8 ± 3.6 vs 3.1 ± 2.1, P = 0.01; bone erosion, 13.5 ± 9.6 vs 4.4 ± 3.6, P < 0.001).ConclusionPersistent subclinical inflammations were shown in patients with sustained remission; BME in MRI may be a strong predictor of future radiographic progression of bone erosions in patients with persistent clinical remission.

DOUBLE PORPHYRIA: LITERATURE REVIEW AND ANALYSIS OF CLINICAL OBSERVATION

A brief review of the literature on the problem of double porphyria and analysis of its own observation is presented. For more than 10 years patient B was observed for more than 10 years with a verified diagnosis of acute intermittent porphyria, which manifested with acute pain abdominal syndrome, neurological disorders in the form of peripheral polyneuropathy and hemiparesis of lower extremities, and hypertension syndrome was also noted. The observed clinical symptoms corresponded to an acute porphyrin crisis in the manifestation and / or relapse of acute intermittent porphyria. The diagnosis was confirmed by a quantitative determination of the excretory profile of porphyrin precursors (δ-aminocaproic acid, porphobilinogen) and porphyrin fractions (uroporphyrin, coproporphyrin). Their concentrations are significantly (especially porphyrin precursors) exceeding the control values, which is the cardinal diagnostic criterion of acute intermittent porphyria. Against the backdrop of persistent clinical and biochemical remission of acute intermittent porphyria, symptoms of photosensitization of the skin (blisters, erosion, pigment spots) on the dorsal surface of the hands began to appear in 4 years. Later, hypertrichosis was formed in the temporo-periorbital region. The constellation type of the excretory profile of porphyrins began to change. Against the backdrop of persistent increased excretion of porphyrin precursors (δ-aminolevulinic acid and porphobilinogen), a progressive increase in the excretion of the fraction of uroporphyrin was observed, which became dominant (up to 58% of the total content of porphyrins). Such a prolonged observation in the dynamics allowed us to state the appearance of a new variant of the porphyrin exchange disturbance, which, taking into account clinical symptoms, corresponded to another form of hepatic porphyria, namely, late cutaneous porphyria. The clinical and biochemical changes in the excretory profile of the parameters of porphyrin metabolism registered in the dynamics of observation may indicate the occurrence of a combined enzymatic defect characteristic of double porphyria. In our case, a manifestation of late cutaneous porphyria was noted against a background of compensated acute intermittent porphyria.

EXTERNAL THERAPY OF ATOPIC DERMATITIS IN CHILDREN

Atopic dermatitis (Ad) is a multifactorial skin disease. As shown by recent studies, a significant role in the pathogenesis of atopic dermatitis is a disturbance of the structural elements of the skin barrier, which contributes to skin xerosis, increase of its permeability to allergens and development of chronic inflammation. As a result, an allergic inflammation develops in the skin. Accordingly, in the treatment of atopic dermatitis great attention should be paid to effective external therapy. In the period of exacerbation of the skin process effective and safe external preparations are drugs of activated zinc pyrithione (APTS) - a line of Skin-Cap products. Use of APTS in atopic dermatitis in children leads to a significant reduction in the severity of inflammation, reduction of the area of skin lesions and severity of symptoms, reducing the need for additional administration of antihistamine and anti-inflammatory drugs, allowing you to reach persistent clinical remission.

Strategy for managing patients with severe cases of rosacea

The article presents 3 clinical observations of women of 51, 55 and 57 years old with severe rosacea, who reached the state of remission after a prolonged course of systemic therapy with low doses of isotretinoin. Cumulative dose of isotretinoin for persistent clinical remission was 46.4 mg/kg of weight, 17.6 mg/kg, 12.6 mg/kg respectively. Difficulties related to the subjective perception of patients of his condition has limited the possibility of increasing daily dose of isotretinoin, and the lack of result from the previous therapy with basic medications has made isotretinoin a drug of choice.

First experience of electroconvulsive therapy in Belarus

Electroconvulsive therapy (ECT) – a method of psychiatric and neurological treatment in which a large epileptiform seizure caused by passing an electric current through the brain of the patient in order to achieve a therapeutic effect. Clinical research of efficiency of application ECT with anesthesia, muscle relaxation and hardware analysis of EEG in patients with resistant forms of mental and behavioral disorders was held in the Republic of Belarus. The duration of the course of ECT, the frequency of sessions, as well as the method of applying electrodes for stimulation were determined by the doctor conducting the sessions of ECT. Sessions were conducted with general anesthesia with the use of muscle relaxants. Analysis of EEG data (Average Seizure Energy Index [ASEI]; Postictal Suppression Index [PSI]; COH–Maximum Sustained Coherence), on the basis of which the dose of stimulation was corrected, was conducted to assess the duration and quality of convulsive seizure during ECT. Significant clinical improvement in patients who underwent ECT sessions was achieved as a result of the study. Application of the method allowed to achieve persistent clinical remission in a short time. The clinical study showed that the use of ECT with anesthesia, muscle relaxation and hardware analysis of the EEG in the Republic of Belarus may be effective against drug-resistant forms of mental and behavioral disorders.

Igg4-related sclerosing disease of the respiratory tract: case report and literature review

IgG4­related sclerosing disease (IgG4­RSD) is a recently described immunodependent system pathology, characterized by focal or diffuse inflammatory infiltration of the affected organs and tissues by plasma cells expressing IgG4, and the subsequent development of obliterate phlebitis and the relevant organs fibrosclerosis, it is accompanied by the increased levels of IgG4 in blood serum. According to recently published materials, the disease can also occur in the respiratory system. In the article, the authors describe the first documented case of IgG4­RSD with isolated lesions of the larynx. The diagnosis was established by a comprehensive examination with the exception of system failures, determination of the IgG4 level in blood serum and immunohistochemical analysis of histological preparations with coloring to IgG4­secreting plasma cells. After establishing the diagnosis, the patient underwent a course of systemic hormone therapy. Now the patient has persistent clinical and radiological remission of the disease.

P93 – 2953: Identification of a new mutation of the SCL1A gene in a patient with GLUT1-DS

Objective Diagnostic assessment of a boy of 13 years old. From the age of 16 months, he has showed an isolated oculomotor disorder associated with a delay in acquisition of psychomotor milestones. Furthermore, at 4 years old, he presented absence-like seizures, for whom he has been favourably treated with valproic acid. After 9 years of wellness, when antiepileptic drug was stopped, an effort-related paroxysmal myoclonic dystonia appeared, involving mostly lower limbs. Such episodes have rapidly increased in frequency, becoming truly debilitating for the patient. Methods Suspecting a deficit of the enzyme GLUT1, the patient underwent lumbar puncture, video-EEG and molecular genetic analysis. Physical-chemical analysis of CSF demonstrated a glycorrhachia of 45 mg/dL, lactate at the lower limit of the normal range and a CSF/serum glucose ratio of 0.52. Video-EEG excluded epileptic paroxysms. Results The molecular investigation revealed a point mutation not previously described in literature (c.1199G>A; exon 9 – gene SLC2A1); this mutation was absent in parental alleles and thus it was originated de novo. Conclusion After diagnosis, the patient restarted therapy with valproic acid and began to follow strictly ketogenic diet, getting a complete and persistent clinical remission. In conclusion, this report of a GLUT1-DS case is characterized by an unusual presentation and the description of a new causative mutation. More interestingly, it proves the importance of CSF analysis to potentially reveal treatable diseases in children with undiagnosed movement disorders.

Etanercept induces remission of polyarteritis nodosa: a case report.

Etanercept induces remission of polyarteritis nodosa: a case report.

AB0671 Dosage Reduction of Biological Therapy in Patients with Axial Spondyloarthritis in Persistent Clinical Remission

BackgroundThe effectiveness of TNF blockers in treating axial Spondyloarthritis is well-known but there are a few evidence on the dosage reduction in patients in persistent clinical remission (1-4).ObjectivesOur objective was...

Identification of kit(M541L) somatic mutation in chronic eosinophilic leukemia, not otherwise specified and its implication in low-dose imatinib response.

Activating mutations of KIT receptor tyrosine kinase have been reported in different neoplasms. The M541L KIT substitution (KIT(M541L)) has been described to be associated with pediatric mastocytosis, to enhance growth rate of the affected cells and to confer higher sensitivity to imatinib therapy. We investigated the presence of KIT(M541L) in five males with chronic eosinophilic leukemia, not otherwise specified (CEL, NOS), all negative for Platelet-derived growth factor-alpha (PDGFR) or PDGFRbeta abnormalities, which responded to imatinib therapy. To assess whether the mutation was constitutive or somatic in nature, we evaluated its presence analyzing either the neoplastic or normal cell population (epidermal cells or CD3-positive T lymphocytes). KIT(M541L) substitution was found in 4 out of 5 patients and in all it was somatic in nature. All patients were treated with low dose imatinib (100 mg daily orally), achieving complete and persistent clinical and hematological remission (median follow-up 74 months). One patient relapsed after 50 months. Our study strongly suggests to search for the KIT(M541L) in patients with CEL, NOS, negative for PDGFRalpha and PDGFRbeta abnormalities, to identify a subgroup of cases who may benefit from low dose imatinib therapy.

THU0368 Dosage Reduction of Biological Therapy in Patients with Axial Spondyloarthritis in Persistent Clinical Remission.

BackgroundThe effectiveness of TNF blockers in treating axial Spondyloarthritis patients is well know, but we don’t know most appropriate dosage for treatment of patients in persistent clinical remission.ObjectivesOur objective is...

FRI0065 Residual power doppler signals during clinical remission determine joint prognosis in rheumatoid arthritis

Background“Clinical remission” has been a realistic goal of treatment in rheumatoid arthritis (RA). However, there is evidence that subclinical synovitis is associated with progression of structural damage even after achieving...

Palmar Fasciitis and Polyarthritis Syndrome in Patients with Ovarian Cancer—A Case Report and Review of the Literature

Palmar Fasciitis and Polyarthritis Syndrome in Patients with Ovarian Cancer—A Case Report and Review of the Literature

Long-term Successful Adalimumab Therapy in Severe Hidradenitis Suppurativa

Several studies report the use of tumor necrosis factor alpha (TNF-alpha) inhibitors in refractory hidradenitis suppurativa (HS), particularly infliximab and etanercept. However, very limited data have been reported for adalimumab, the newest fully human anti-TNF-alpha monoclonal antibody. We evaluated the long-term efficacy and safety of adalimumab therapy in 6 patients with refractory HS. In the case of positive culture findings from any draining lesion, antibiotic therapy was administered for at least 2 weeks before initiating adalimumab therapy. Adalimumab (in 40-mg subcutaneous injections) was prescribed every other week. If the disease was inadequately controlled, the dosage was increased to 40 mg/wk. If HS was in persistent clinical remission, adalimumab therapy was gradually decreased to 40 mg every 3 weeks. Quality of life was assessed using the Dermatology Life Quality Index. Six patients (mean [SD] age, 39.3 [12.9] years) with severe HS (mean [SD] duration, 22.5 [11.7] years) were treated with adalimumab. Significant improvements after 1 month of treatment were seen in the Dermatology Life Quality Index; in the number of affected regions, nodules, and fistulas; and in the basic laboratory findings. Improvements were maintained for a mean (SD) follow-up of 21.5 (7.1) (range, 13-29) months. Adalimumab was well tolerated. Conclusion Adalimumab appears to be an effective and safe treatment for refractory HS.

Immunochemotherapy with in vivo purging and autotransplant induces long clinical and molecular remission in advanced relapsed and refractory follicular lymphoma

BackgroundTo evaluate the clinical outcome of patients with relapsed or refractory follicular lymphoma treated with immunochemotherapy, in vivo purging and high-dose therapy with autotransplant. Patients and methodsSixty-four patients were enrolled in the trial. Primary end point was progression-free survival (PFS). Secondary end points were the in vivo purging effect on stem-cell harvest and the impact of molecular response on the outcome. ResultsAt enrollment, 59% of patients were PCR+ for bcl-2 rearrangement in bone marrow (PCR-informative). After the immunochemotherapy, before mobilization, 97% obtained complete response or partial response and 87% of patients informative for bcl-2 were molecularly negative. Sixty-one patients proceeded to in vivo purging and peripheral blood stem cell (PBSC) mobilization with rituximab and high-dose AraC. The median number of CD34+ cells collected was 16.6 × 106/kg. Of 33 PCR-informative patients, the harvests resulted in PCR- in all. Fifty-eight patients received high-dose therapy and autotransplant of in vivo purged PBSC. After a median follow-up of 3.5 years, 41 patients are in complete remission. Five-year PFS is 59%. ConclusionThis study demonstrates that patients with advanced relapsed or refractory follicular lymphoma treated with immunochemotherapy, in vivo purging and autotransplant may obtain long-lasting PFS. In bcl-2-positive patients, in vivo purging allows the harvest of lymphoma-free PBSC. Absence of the bcl-2 rearrangement after autotransplant is associated with persistent clinical remission.

ＨＩＶ感染症を合併し，シクロスポリン投与を要した抗ＭｕＳＫ抗体陽性・重症筋無力症の１例

A-58-year old man presented with fluctuating ptosis and dysphagia. When he was 53 years old, he developed oral candidiasis and serum human immunodeficiency virus (HIV) RNA was detected. After starting highly active antiretroviral therapy, serum HIV RNA became undetectable. Neurological examination revealed ptosis and bulbar symptoms. Myasthenia gravis was comfirmed by a positive edrophonium test, showing 20% decrement of the compound muscle action potential on repetitive stimulation. Anti-acetylcholine receptor antibodies were negative and anti-muscle specific tyrosine kinase (MuSK) antibodies were positive. The chest CT scan was normal. He experienced transient clinical remission with pyridostigmine bromide and prednisolone. However relapse occurred after he returned to work. Persistent clinical remission was first observed after cyclosporin administration. There are eleven reports in which patients had concomitant myasthenia gravis and HIV infection. Most of those cases were benign in clinical course and required only anticholinesterase therapy. In our case, however, anti-MuSK antibodies were positive, and symptoms of myasthenia gravis remained despite prednisolone administration. Cyclosporin is directly active against HIV, and thus, cyclosporine therapy may be helpful in patients with concomitant myasthenia gravis and HIV infection.

Argon plasma coagulation for radiation proctitis

Background. Rectal blood loss is a common late sequel of radiation proctitis. Teleangiectasias appear in the mucosa in 2–5% of patients after radiotherapy of the pelvis. Since pharmacotherapy is usually not beneficial, local treatment modalities with formalin irrigation, Nd:YAG laser and argon plasma coagulation (APC) have been advocated, but experience is still limited. Methods. Between January 1997 and August 2001, 50 consecutive patients with rectal bleeding due to radiation proctitis were included for treatment with APC. Thirteen patients suffered from anaemia, six of whom required blood transfusion. Nine patients were receiving anticoagulant therapy and 10 patients used low-dose aspirin. APC was performed, applying the no-touch spotting technique at an electrical power of 50 Watt and an argon gas flow of 2.0 l/min. Pulse duration was less than 0.5 s. Treatment sessions were carried out at intervals of 3 weeks. Results. In 47 out of 48 patients (98%) in whom the effect could be assessed, APC led to persistent clinical and endoscopic remission of rectal bleeding after a median of three sessions. One patient developed recurrent blood loss after resuming anticoagulant therapy for his aortic valve prosthesis. No adverse effects were encountered after initial treatment. One serious complication occurred in a patient with recurrent blood loss when he was prescribed aspirin for a transient ischaemic attack 2 years after the initial APC. Re-treatment resulted in a major rectal bleeding from a small ulcer with a visible vessel. Conclusions. APC is a safe, effective and well-tolerated treatment for blood loss due to radiation proctitis. The use of anticoagulants and aspirin seems to be a co-factors that induces bleeding.

Blood Flow Cytometry Allows Us To Dispense with Bone Marrow Biopsy in the Evaluation of Treatment Response in CLL.

Aims: To evaluate the diagnostic value of bone marrow biopsy (BMB) with immunohistochemistry studies compared to blood flow cytometry (FC) in response evaluation after treatment in CLL. Patients and methods: 75 previously untreated patients with stage B and C-CLL were treated with oral Fludarabine (30mg/m2/d) + Cyclophosphamide (200mg/m2/d) combination, day 1 through 5, repeated every 28 days for 6 courses. Response evaluation was performed 2 months after the completion of therapy, according to NCI criteria. Sixty patients out of 75 achieved partial or complete remission and underwent BMB. Minimal residual disease (MRD) was simultaneously evaluated on paraffin-embedded BMB by anti-CD5, -CD20 and -CD3 immunostaining and in the peripheral blood using a very high sensitive FC method (Maloum et al, Br J Haematol, 2002, 119:970–5). BMB immunostaining was considered positive for MRD in case of concurrent expression of CD20 and CD5 by the same cells assessed on consecutive sections. Twenty nine patients could have been analyzed so far. The median follow-up was of 49 months [range 19–56].Results: In all these 29 patients, BMB was considered histologically normal according to the NCI criteria. In 23/29 cases, bone marrow immunostaining did not show expressing CD20 cells. Fifteen out of 23 were in phenotypic remission using blood FC (PhR), of whom only 2 relapsed (at 31 and 37 months). The remaining 8/23 patients did not achieve PhR and all relapsed (at 22, 27, 37, 40, 45 and 51 months). In 6/29 patients, immunochemistry on BMB showed persistent B CD5+ cells : 3 did not achieve PhR of whom 2 relapsed (at 32 and 37 months) while 3 were in complete PhR and displayed a persistent clinical complete remission with a follow-up of 39, 47 and 47 months respectively. Interestingly, in the latter 3 cases, FC analysis showed an expansion of polyclonal BCD5+ cells in blood as assessed, which could explain the presence of such normal BCD5+ cells in the bone marrow. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and diagnostic accuracy (ACC) to predict relapse for BMB and FC are given in the following table:Sensitivity (%)Specificity (%)PPV (%)NPV (%)ACC (%)BMB16.776.533.356.351.7FC83.394.190.988.989.7Conclusion: These results strongly suggest that BMB should not be indicated in the evaluation of treatment response and is not useful for relapse prediction. In contrast, blood flow cytometry constitutes the best method in routine laboratory to predict clinical outcome and should be included in response treatment criteria in CLL.

Helicobacter pylori eradication for the treatment of low-grade gastric MALT lymphoma: Follow-up together with sequential molecular studies

Helicobacter pylori infection is associated with low-grade gastric MALT lymphoma, and available data support that the eradication of the H. pylori can cause histological regression of the lymphoma. Nine patients with low-grade gastric MALT lymphoma were treated with amoxicillin, metronidazole, and omeprazole for 14 days in a prospective study. Patients were followed up with sequential endoscopy, mapping gastric biopsies, and molecular studies with PCR amplification of the IgH gene in order to assess the response to H. pylori eradication and the evolution of the histological molecular responses. H. pylori was eradicated in all patients and reinfections were not demonstrated. After H. pylori eradication treatment, the lymphoma regressed both endoscopically and histologically in eight of the nine patients (88.8%). In four of the eight histologically cured patients, no clonal band was detected by PCR; in the remaining four patients; PCR identified a clonal band, which disappeared in all patients after a mean of 12 +/- 4 months. No clonal band was detected by the PCR analysis in any of the eight patients with histological regression after a median of 7 +/- 6 months (range 1-20). The seven followed-up patients have a persistent clinical and histological remission after a median of 14 +/- 5 months. (1) Low-grade gastric MALT lymphoma can be histologically cured with eradication therapy for H. pylori. (2) After histological regression, PCR amplification of the IgH gene can identify an eventually persisting clonal population. (3) Sequential histological and molecular studies are essential for the assessment of the evolution of the lymphoma. (4) The clonal population tends to disappear, but its disappearance may be delayed for months. (5) Patients with histological regression but with a persistent clonal band should not be treated unless the lymphoma can be histologically demonstrated. All these observations suggest that gastric lymphoma can be effectively cured, but the ultimate fate of these patients is unknown until long-term follow-up studies are available.