Buerger’s disease: a 10-year experience in Tehran,Iran

The clinical spectrum of Buerger’s disease (TAO; thromboangiitis obliterans) seems to be changing. Considering the lack of any formal studies on the epidemiology of this disease in Iran, we evaluated the data of 198 confirmed cases of TAO during the period 1996–2006. Diagnosis was based on Papa’s scoring system. All patients were male and aged at the time of diagnosis 40.5 ± 10.1 years. All patients were smokers for a duration of 22.2 ± 11.6 years. There was a strong seasonal variation for admission with winter admissions being significantly the most common (P < 0.001). Patients, 181 (91.4%), had only lower limb involvement, 4 (2.0%) had solely upper limb disease, and 13 (6.6%) suffered from both. Sympathectomy, amputation, and bypass graft surgery were performed in 97 (49.0%; lumbar: 92, thoracic: 5), 71 (35.9%), and 28 (14.1%) patients, respectively. The present report is the second one to show seasonal variation in TAO.     

Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?

Castleman disease (CD) is a rare lymphoproliferative disorder that can be unicentric or multicentric. Multicentric CD (MCD) is further subdivided into human herpesvirus type-8-associated, POEMS syndrome-associated, and idiopathic (iMCD). TAFRO syndrome is a newly identified disorder of unknown etiology characterized by t hrombocytopenia, a nasarca, f ever, r eticulin myelofibrosis, renal dysfunction, and o rganomegaly. The TAFRO syndrome is sometimes regarded as a subtype of iMCD (TAFRO-iMCD), whereas iMCD without TAFRO syndrome is considered “not otherwise specified” (iMCD-NOS). However, a proportion of patients with TAFRO syndrome have been diagnosed without lymph node biopsies (TAFRO syndrome without proven iMCD; TAFRO-w/op-iMCD). To clarify the clinical features of iMCD-NOS, TAFRO-iMCD, and TAFRO-w/op-iMCD, we retrospectively analyzed 220 patients extracted from the database of the Multicenter Collaborative Retrospective Study for Establishing the Concept of TAFRO Syndrome. The patients included 87 with iMCD-NOS, 63 with TAFRO-iMCD, and 19 with TAFRO-w/op-iMCD. Patients in all three groups exhibited anemia, hypoalbuminemia, and elevated serum C-reactive protein and interleukin-6 levels. No significant differences in clinical, laboratory, and prognostic features were noted between the TAFRO-iMCD, and TAFRO-w/op-iMCD groups. However, the iMCD-NOS group exhibited polyclonal hyper-γ-globulinemia. The five-year survival rates of patients in the iMCD-NOS and TAFRO-involved groups were 100% and 66.5%, respectively (dropping markedly during the first few months in the latter). The iMCD-NOS and the TAFRO-iMCD samples typically showed plasma cell and mixed-type histologies, respectively. Thus, iMCD can be classified into two distinct subtypes, iMCD-NOS and TAFRO-iMCD. As such, TAFRO-iMCD and TAFRO-w/op-iMCD may be considered the same entity, requiring prompt diagnosis and intensive care.     

A case of TAFRO syndrome,a variant of multicentric Castleman’s disease,successfully treated with corticosteroid and cyclosporine A

We report a case of a 46-year-old woman with fever, pleural effusion, massive ascites, severe edema, hepatosplenomegaly, elevation of serum creatinine level, proteinuria, and severe thrombocytopenia. Her clinical features were compatible with TAFRO syndrome proposed as a variant of multicentric Castleman’s disease, that is occasionally associated with poor prognosis. Treatment with corticosteroid improved her symptoms partially. However, thrombocytopenia, ascites, and edema persisted. The use of cyclosporine A successfully improved her condition, resulting in remission.     

Myocarditis as a form of relapse in two patients with adult Still’s disease

Still’s disease is a subset of juvenile idiopathic arthritis (JIA) that usually presents with intermittent fever, rash, and arthritis. Extra-articular flares can occur several years after disease onset. We report two cases of adult Still’s disease with myocarditis after several years of being in remission. A 34-year-old Caucasian man with history of systemic juvenile arthritis in remission since age 13 was admitted in hospital with 10 days history of fever, odynophagia, and arthralgias. Chest X-ray and cardiac ultrasound showed cardiac enlargement. An endomyocardial biopsy revealed acute myocarditis. He was treated with methylprednisolone and intravenous gammaglobulin, with improvement of his general condition and cardiac parameters. A 16-year-old Caucasian male patient with history of systemic JIA in remission for the last 7 years was admitted with 7 days history of fever, odynophagia, arthralgias, and myalgias. Two days after admission, he developed chest pain and pericardial rubbing was found on examination. Cardiac ultrasound showed left ventricular dilatation with impaired systolic function, and posterior, inferior and apical-septal wall hypokinesia. Blood test showed elevated creatine phosphokinase levels. He was treated with IV methylprednisolone with normal follow-up cardiac ultrasound. Cardiac involvement in patients with systemic JIA can be the first symptom of disease reactivation, even after many years of disease remission.     

Macrophage activation syndrome in Still’s disease: analysis of clinical characteristics and survival in paediatric and adult patients

Clinical Rheumatology - Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, complicating Still’s disease, both in paediatric and adult patients. In...     

Pasireotide monotherapy in Cushing’s disease: a single-centre experience with 5-year extension of phase III Trial

Purpose

A recent phase III randomized controlled trial (NCT00434148) showed efficacy of pasireotide in the treatment of patients with Cushing’s disease (CD). Patients were invited to participate in an extension phase of the protocol and a subgroup had a sustained response. We report the experience with 4 patients in our center of which 2 full responders have completed 5.5 and 4.25 years of treatment with disease control.

Methods

The trial protocol was described previously. The extension phase consisted of 3-monthly visits with clinical, biochemical, and imaging evaluation and investigator-driven pasireotide titration. Research charts were retrospectively analyzed.

Results

Four patients with persistent CD following pituitary surgery completed the first 6 months of the trial and 3 continued in the next 6 month open-label phase. Two patients with baseline urinary free cortisol (UFC) 5.3–6.7 times the upper limit of normal had a rapid sustained response to pasireotide and entered the extension phase after 12 months. They remain in clinical and biochemical disease remission and 1 patient now only requires 300 μg daily of pasireotide. All 4 patients developed glucose intolerance; however, the two patients in the extension phase were eventually able to discontinue all diabetes pharmacotherapy. Adverse events included second degree atrioventicular block type 1 without QT prolongation in a patient with pre-existing sinus bradycardia, and symptomatic cholelithiasis requiring cholecystectomy in a second patient.

Conclusions

Pasireotide therapy can provide normalization of UFC and of clinical symptoms and signs of CD during up to 5 years of follow-up. This study demonstrates the possible recuperation of normoglycemia after continued use of pasireotide and control of underlying hypercortisolemia. Longer-term monitoring for potential adverse events related to continued use of pasireotide is indicated.     

Castleman’s disease of the spleen

Castleman's disease(CD) is a rare lymphoproliferative disorder of unknown etiology.Clinically,it occurs as a localized(unicentric) disease or as a systemic(multicentric) disease.Unicentric Castleman's disease(UCD) presents as a solitary mass and primarily affects the mediastinal,retroperitoneal,and cervical lymph nodes.In contrast to multicentric CD,which involves peripheral lymphadenopathy and numerous systemic symptoms,UCD is not typically associated withgeneralized symptoms.Three main distinct histologic variants are recognized:hyaline-vascular type,plasma cell type,and mixed type.Extranodal CD is rare.Specifically,UCD exclusively in the spleen is extremely rare,with only 2 cases described in the literature to date.Here,we describe an asymptomatic 75-yearold man with a 5.7 cm × 4.5 cm sized heterogenous enhanced mass located in the spleen.He underwent surgical resection for diagnosis and treatment.A pathologic examination indicated the hyaline-vascular type of CD.In this patient,the preoperative diagnosis was difficult to determine,and therefore,invasive procedures were required.     

Behcet’s disease and cardiovascular involvement: our experience of asymptomatic Behcet’s patients

Behcet’s disease is generally defined by oral and genital ulcers and uveitis. It is also known as a recurrent multisystemic and inflammatory disease. It is mostly seen in Mediterranean countries and the Far East.The aetiology of Behcet’s disease is associated with viral, toxic, bacterial and immunological factors. It was defined in 1963 as an auto-immune disease caused by auto-antibodies against the oral mucosa. Vascular involvement is 2–7% and it is usually seen in patients between the ages of 20 and 40 years.Behcet’s disease is a non-specific arterial and venous vasculitis.1-8 Proximal and distal anastomotic aneurysm formation after surgery is not rare one to 12 months postoperatively. Recurrent surgical interventions increase the risk of mortality and morbidity.9,10 Cardiovascular involvement in Behcet’s disease includes pericarditis, coronary arterial disease, cardiomyopathy and valvular dysfunction.11 The aim of this study was to report our experience of cardiovascular involvement with asymptomatic Behcet’s disease.     

Multicentric Castleman’s disease complicated by tumor lysis syndrome

We report a case of Castlemans disease that developed tumor lysis syndrome spontaneously and after systemic chemotherapy. A 44-year-old male patient was admitted with a 2-week history of abdominal distension accompanied by dyspnea. Physical examination revealed multiple lymph node enlargements. After admission, spontaneous hemoperitoneum developed and he underwent exploratory laparotomy, with the removal of the ruptured spleen. Pathologic review of the splenic tissue and excised lymph node gave the diagnosis of multicentric Castlemans disease. He experienced two episodes of tumor lysis syndrome, initially spontaneous and then chemotherapy related, which needed vigorous management including hemodialysis and intensive fluid therapies. To our knowledge, this is the first reported case of Castlemans disease complicated by tumor lysis syndrome. This suggests that the possibility of tumor lysis syndrome should be considered when treating Castlemans disease with a large disease burden.     

Prevalence of Behcet’s disease among adult patients consulting three major clinics in a Druze town in Israel

To evaluate the prevalence of Behçet’s disease (BD) in a Druze community in Israel, we conducted a two-stage clinic-based survey in an Israeli Druze town. The first stage aimed to identify patients with recurrent aphthous stomatitis (RAS) in all patients who visited three of the largest clinics in the town during a period of 6 months. The second stage aimed to identify those patients with RAS who fulfilled the diagnostic criteria for BD according to the International Study Group (ISG) criteria. One thousand and eighty-three out of about 4,000 registered subjects were interviewed, 63 of whom had RAS (5.8%). Two patients fulfilled the ISG criteria for BD, resulting in a calculated prevalence in the range of 2:1,083–2:4,000, i.e., 50–185:100,000. Another two patients with oral and genital aphthosis but without eye or skin lesions were diagnosed as suspected BD. The very high prevalence of BD, as found in our study, places the Druze among the populations with the highest prevalence of the disease all over the world, though selection biases could account for overestimation as well as underestimation of the actual BD prevalence. Our findings call for genetic studies to explore whether there is a genetic predisposition to BD in this population.     

Multi-organ failure in adult onset Still’s disease: a septic disguise

Juvenile dermatomyositis (JDM) is a rare disease, and Sjögren’s syndrome (SS) is unusual in adolescents. We report the first case of biopsy-proven JDM and SS with pulmonary involvement. A 15-year-old adolescent boy presented with recurrent parotid gland hypertrophy, severe muscle weakness, pronounced skin rash and widespread lymphadenopathy. JDM was diagnosed by clinical examination, elevated muscle enzymes, electromyography and muscle biopsy; SS was diagnosed by xerostomia, anti-Ro (SS-A) positivity and histopathological analysis of salivary gland tissue. This case illustrates a systematic approach which we feel is especially important in the younger patient with a more plastic immune system.     

Multi-organ failure in adult onset Still’s disease: a septic disguise

The diagnosis of adult onset Still’s disease is difficult in the absence of definite clinical and laboratory criteria. A delayed diagnosis of adult onset Still’s disease was made in a 23-year-old female who developed multi-organ failure and disseminated intravascular coagulation with fingertip auto-amputation during a febrile illness considered septic due to the persistence of elevated serum procalcitonin concentration.     

Evolution of disease phenotype in adult and pediatric onset Crohn’s disease in a population-based cohort

AIM:To investigate the evolution of disease phenotypein adult and pediatric onset Crohn’s disease(CD) populations,diagnosed between 1977 and 2008.METHODS:Data of 506 incident CD patients were analyzed(age at diagnosis:28.5 years,interquartile range:22-38 years).Both in-and outpatient records were collected prospectively with a complete clinical follow-up and comprehensively reviewed in the population-based Veszprem province database,which included incident patients diagnosed between January 1,1977 and December 31,2008 in adult and pediatric onset CD populations.Disease phenotype according to the Montreal classification and long-term disease course was analysed according to the age at onset in time-dependent univariate and multivariate analysis.RESULTS:Among this population-based cohort,seventy-four(12.8%) pediatric-onset CD patients were identified(diagnosed ≤ 17 years of age).There was no significant difference in the distribution of disease behavior between pediatric(B1:62%,B2:15%,B3:23%) and adult-onset CD patients(B1:56%,B2:21%,B3:23%) at diagnosis,or during follow-up.Overall,the probability of developing complicated disease behaviour was 49.7% and 61.3% in the pediatric and 55.1% and 62.4% in the adult onset patients after 5-and 10-years of follow-up.Similarly,time to change in disease behaviour from non stricturing,non penetrating(B1) to complicated,stricturing or penetrating(B2/B3) disease was not significantly different between pediatric and adult onset CD in a Kaplan-Meier analysis.Calendar year of diagnosis(P = 0.04),ileal location(P < 0.001),perianal disease(P < 0.001),smoking(P = 0.038) and need for steroids(P < 0.001) were associated with presence of,or progression to,complicated disease behavior at diagnosis and during follow-up.A change in disease location was observed in 8.9% of patients and it was associated with smoking status(P = 0.01),but not with age at diagnosis.CONCLUSION:Long-term evolution of disease behavior was not different in pediatric-and adult-onset CD patients in this     

Coronavirus disease 2019 in patients with Behcet’s disease: a report of 59 cases in Iran

Clinical Rheumatology - To present the clinical characteristics, disease course, management, and outcomes of COVID-19 infection in patients with Behcet’s disease (BD). In this retrospective...     

Surgical outcomes in patients with Cushing’s disease: the Cleveland clinic experience

Context

Transsphenoidal surgery (TSS) to resect a pituitary adenoma is considered first-line treatment for patients with Cushing’s disease (CD). Early, post-operative remission rates >80% are expected for patients with a microadenoma (≤?10 mm) visible on magnetic resonance (MR) imaging.

Objective

To report surgical outcomes and predictors of remission in a specialist center for patients with CD.

Patients and methods

Clinical data was obtained from a prospective CD database in addition to review of all electronic medical, laboratory and surgical patient records. Patients who underwent their first TSS by one neurosurgeon between 2004 and 2013, and had a minimum 1 year follow up, were evaluated.

Results

One hundred and one consecutive patients with CD (73F, 28M) underwent TSS. Median (range) age and follow-up were 47 (15–87) and 4.33 (1–9.8) years, respectively. At surgery, 74 (73.2%) patients had a microadenoma, 27 a macroadenoma; six of the latter patients had a planned, subtotal resection to control neurological signs due to mass effect. Initial remission rates were: microadenoma, 89% (66/74); macroadenoma, 63% (17/27); and 81% (17/21) in those macroadenomas where complete surgical removal was anticipated. Initial non-remission occurred in 18 patients, ten macro- and eight microadenoma; six of 18 had residual disease on most recent follow up. Six (2 macro, 4 micro) of the 83 patients with initial remission have had late (>12 months) recurrence of hypercortisolism that required either repeat TSS or adjunctive therapy, three of whom have persistent hypercortisolism. Macroadenoma (p?=?0.003) and tumor invasion beyond the pituitary and sella (p?<?0.001) were associated with failure to obtain remission with the initial TSS and greater likelihood of late recurrence. Patients in whom no lesion was seen on neuroimaging had rates of initial remission (21/25 or 84%) and a similar late recurrence rate of 4% (1/25) in comparison with those with MR-visible microadenomas (3/49, or 6%).

Conclusions

A team-based approach, in a specialized pituitary center, can lead to initial and durable, long-term remission in patients with CD. The presence of a macroadenoma and tumor extension beyond the pituitary and sella were predictive of initial non-remission as well as risk of late recurrence.

     

Clinical characteristics of pulmonary artery involvement in patients with Behçet’s syndrome: single-centre experience of 61 patients

Clinical Rheumatology - To report the clinical characteristics of pulmonary artery involvement (PAI) in patients with Behçet’s syndrome (BS) and to define the predictors of relapses. We...     

Risk factors of disease activity in patients with Behçet’s syndrome

Clinical Rheumatology - To investigate the clinical characteristics and laboratory data in Behçet’s syndrome (BS) patients in China and analyze the risk factors of disease activity. A...     

Treatment of anal fistula with FiLaC®: results of a 10-year experience with 175 patients

Techniques in Coloproctology - Treatment of fistula-in-ano with fistula laser closure (FiLaC®) is a sphincter-saving procedure indicated for patients with complex anal fistulas. The aim of our...