Patients with chronic thromboembolic pulmonary hypertension (CTEPH) have poor prognosis, and pulmonary endarterectomy (PEA) is considered the treatment of choice for this condition. We report a case and review the literature of successful PEA for CTEPH due to antiphospholipid syndrome associated with systemic lupus erythematosus. The definitive and decisive approach ...

A 55-year-old Japanese female was diagnosed with systemic lupus erythematosus (SLE) and developed nephrotic syndrome. She was diagnosed with lupus nephritis by a percutaneous renal biopsy. She was treated with intravenous steroid pulse therapy twice, but it proved to be ineffective. She achieved a complete remission after intravenous cyclophosphamide pulse ...

We report on an 11-year-old girl who developed steroid-resistant nephrotic syndrome (NS) at the onset of systemic lupus erythematosus (SLE), and clinical and renal histological findings suggested that her NS would be associated with SLE-related podocytopathy. Although initial treatment with intravenous pulse methylprednisolone was ineffective, following treatment with cyclosporine and ...

Background  Overlap syndromes represent disorders that combine diagnostic criteria of two or more different connective tissue diseases. Methods  We herein describe the case of a 34-year-old patient. Results  Our patient developed a lesion on the scalp and forehead following Blaschko's line typical for linear morphea "en coup de sabre", while ...

Immunoglobulin M (IgM) nephropathy is an uncommon glomerular disease characterized by IgM deposits in the mesangium. This case report describes a 52-year-old woman with a 10-year history of underlying systemic lupus erythematosus with minimal proteinuria who developed sudden onset of nephrotic syndrome. Renal biopsy revealed IgM nephropathy, with no clear ...

To describe a case of propylthiouracil-induced lupus, complicated with antiphospholipid syndrome and acute ischemic stroke. Case report. Academic medical center. Patient  A 27-year-old man with a diagnosis of Graves disease developed multiple ischemic strokes 2 weeks after starting treatment with propylthiouracil. Thyrotoxicosis and abnormal hypercoagulable and rheumatological profiles were remarkable, ...

A 16-year-old male with severe thrombocytopenia and progressive multiple organ infarctions was diagnosed as having catastrophic antiphospholipid syndrome (CAPS) complicated with systemic lupus erythematosus, and was successfully treated with combination of anticoagulants, corticosteroids, plasma exchange, and intravenous cyclophosphamide. Antibodies to phosphatidylserine/prothrombin (PS/PT) complex and cardiolipin (CL)/β(2)-glycoprotein I (β(2)GPI) were simultaneously detected, ...

We report a case of a 13-year-old female presenting with deep venous thrombosis in her left lower limb. Investigations led by her symptoms and history revealed persistently positive lupus anticoagulant and anticardiolipin antibodies, indicating the diagnosis of antiphospholipid syndrome. Double-stranded DNA antibodies were detected positive pointing to the coexistence of ...

BACKGROUND: The occurrence of erythema multiforme (EM)-like lesions in association with lupus erythematosus (LE) is often referred to as "Rowell syndrome" (RS). However, the existence of RS, or at least its nosographic independence from LE, is questioned. The association of Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) with LE is also ...

OBJECTIVES: Several case reports of lupus-like syndrome suggest that statins could have triggered the development of this rare autoimmune disease. However, data on the association between statin use and lupus-like syndrome are scarce. We assessed whether there was an association between statin use and the occurrence of lupus-like syndrome. METHODS: ...

OBJECTIVE: To determine the prevalence of systemic sclerosis (SSc) overlap syndrome and autoantibody profile in a large single-center cohort. METHODS: SSc diagnoses, subsets, and autoantibody profiles were obtained from clinical records of patients attending the Centre for Rheumatology, Royal Free Hospital, between September 1999 and February 2007. RESULTS: In total, ...

Systemic lupus erythematosus (SLE) and small-sized vessel vasculitis are usually two distinguishable autoimmune diseases. However, a vasculitis may be found in the course SLE but rarely corresponds to an ANCA-associated vasculitis (AAV). We report four cases of de novo SLE associated with AAV, our aim being to discuss the clinical ...

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by recurrent thrombotic events, miscarriages and thrombocytopenia with persistently positive antiphospholipid antibodies.(1,2) APS may be isolated (primary APS) or associated to a connective tissue disease, most often systemic lupus erythematosus (SLE).(1,2) APS usually affects young patients before the fifth decade(3) with stroke ...

One of identical twin girls was born with ulcers on her leg, and shortly after birth developed a flaccid blister on the leg. Subepidermal blister with vacuolar degeneration of basal cell layer and the heavy infiltration of mononuclear cells in the upper dermis were observed in the blister lesion. She ...

Hemophagocytic syndrome (HPS) is an unusual disorder associated with systemic lupus erythematosus (SLE). A 64-year-old woman was admitted because of fever and urticarial vasculitis. Laboratory data revealed pancytopenia and immunological abnormalities, suggesting elevated disease activity. Prednisolone monotherapy failed to improve the pancytopenia despite the amelioration of other clinical findings. Because ...

Intestinal pseudo-obstruction (IpsO) occurs in a small subgroup of patients with systemic lupus erythematosus (SLE), and has been under-recognized. The etiology and mechanisms are unclear. Herein, we report two cases. In case 1, IpsO occurred as the complication in a previously diagnosed SLE patient. In case 2, IpsO with an ...

Aicardi-Goutières syndrome is an early-onset encephalopathy with a presumed immune pathogenesis caused by inherited defects in nucleic acid metabolism. The clinical picture resembles a congenital viral infection despite negative investigations for common viruses. In addition to leukoencephalopathy with calcifications of basal ganglia, patients show increased levels of the antiviral cytokine ...

Anti-Ro autoantibody is associated with Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), and neonatal lupus syndrome (i.e., congenital complete heart block in newborns). Generally, the adult atrioventricular (AV) node is believed to be relatively resistant to the scarring effects of anti-Ro/anti-La autoantibodies. However, there have been some reports of adult ...

International Journal of Paediatric Dentistry 2011 Background.  Primary Sjögren syndrome is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around 10 years of age. Diagnosis during childhood is difficult, especially because of the diversity of the clinical presentation and difficulty obtaining reliable history data, accounting ...

Objective: The aim of this study was to evaluate the association between vision and hearing impairment and depressive-anxiety syndrome in a large population participating in the Faenza Project, Northern Italy. Method: The study population consisted of 7389 participants not affected by dementia, 4408 (59.7%), of whom were women, mean age ...

Catatonia is a syndrome of physical and behavioral abnormalities that can result from psychiatric, neurological, or medical illness. Although systemic lupus erythematosus (SLE) is commonly known to cause neurological and psychiatric manifestations, it has only rarely been reported to cause the catatonic syndrome. In nearly all previously reported cases, the ...

Kabuki syndrome is characterized by long palpebral fissures, large ears, a depressed nasal tip, and skeletal anomalies associated with postnatal dwarfism and mental retardation. There have been few prior detailed descriptions of strabismus or stereopsis in these patients. We report a patient with Kabuki syndrome who showed small-angle strabismus and ...

BACKGROUND: Depression is associated with increased physical morbidity and overall mortality. The results of a previous investigation on the relationship of the metabolic syndrome and its single components with coronary heart disease, cardiovascular disease (CVD), and all-cause mortality suggested that the metabolic syndrome is a marker of CVD risk, but ...

To assess correlation between the prognosis and epidemiological, clinical, laboratory, electrophysiological findings in patients with Guillain-Barré syndrome (GBS). We reviewed the medical records of 104 GBS patients who were hospitalized and followed up at our outpatient clinic during October 1997-November 2007. Guillain-Barré syndrome patients were followed up with a median ...

Introduction: The fibromyalgia syndrome (FMS) has a prevalence of about 2% and is characterized by generalized musculoskeletal pain, reduced pain threshold and autonomic and functional symptoms. It is a multifactorial syndrome with four different subgroups exhibiting pathophysiological and psychiatric findings. No precise treatment strategy is currently available for the different ...

OPINION STATEMENT: Fisher syndrome is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia. It is considered a variant form of Guillain-Barré syndrome, which is associated with anti-GQ1b antibodies. During initial examinations of patients, physicians must rule out other neurologic disorders or conditions that resemble Fisher syndrome, such as ...

Calciphylaxis is a rare but life-threatening disease characterized by deposition of calcium within small and medium sized vessels, with subsequent thrombosis, cutaneous ischemia, and necrosis. Because of its systemic nature, calciphylaxis is typically a symmetrical, bilaterally-distributed phenomenon. Here, we present an unusual case of unilateral leg calciphylaxis in the setting ...

Hemophagocytic syndrome (HPS) is a severe, potentially life-threatening disorder characterized by an excessive activation of macrophages, such as may occur in the setting of lupus. A 62-year-old Japanese woman treated with etanercept for rheumatoid arthritis developed persistent fever, cytopenia, coagulopathy, and hyperferritinemia. Simultaneously, lupus-like features including pleuritis, hypocomplementemia, and positive ...

A 24-year-old man was referred to our hospital due to bilateral hilar lymphadenopathy on chest radiography. He had been under medication for aortitis syndrome and Crohn's disease for 12 years. Surgical biopsy from the anterior segment of the left upper lobe and mediastinal lymph nodes was performed under video-assisted thoracoscopy. ...

PURPOSE: Depersonalization syndrome is characterised by a sense of unreality about the self [depersonalization (DP)] and/or the outside world [derealization (DR)]. Prevalence estimates vary widely. Little is known about childhood antecedents of the disorder although emotional abuse is thought to play a role. METHODS: Longitudinal data from 3,275 participants of ...

Immunological involvement of peripheral nervous system in non-Hodgkin lymphoma (NHL) is very rare and it may be difficult to differentiate it from vincristine-induced neuropathy. We report clinical and electrophysiological findings of an 8-year-old male with NHL who developed acute onset fulminant motor sensory autonomic neuropathy during induction chemotherapy which included ...

Serotonin syndrome is a known entity, which occurs with multiple drugs acting on serotonergic receptors. A 73-year-old lady presented with a history of agitation, altered sensorium, and autonomic hyperactivity after starting escitalopram on therapeutic dosage for her depressive syndrome who was on selegiline for her parkinsonism. This syndrome with therapeutic ...

A case of Henoch-Schönlein purpura with Guillain-Barré syndrome in a 3-year-old-girl is presented. This association is extremely rare. During the course of Guillain-Barré syndrome, a decrease in plasma factor XIII activity was noted. When the Guillain-Barré symptoms improved, the factor XIII activity returned to normal. In the present case of ...

Irritable bowel syndrome (IBS) is a symptom complex characterized by recurrent abdominal pain or discomfort, and accompanied by abnormal bowel habits, in the absence of any discernible organic abnormality. Its origin remains unclear, partly because multiple pathophysiologic mechanisms are likely to be involved. A significant proportion of patients develop IBS ...

We describe an adolescent case of Guillain-Barré syndrome (GBS) mimicking acute methotrexate-associated encephalopathy during chemotherapy for lymphoblastic lymphoma. Although initial presentations of hemiparesis and irritability were suggestive of acute encephalopathy, the diminished deep tendon reflexes and subsequent rapid progression to flaccid triparesis with bulbar palsy were consistent with GBS. After ...

A 5-year-old previously healthy boy presented with typical findings of Guillain-Barré syndrome and unilateral tonic pupil. He was placed on mechanical ventilation for 35 days for respiratory failure. Plasmapheresis and two courses of intravenous immunoglobulin therapy were given to the patient, and he experienced stepwise recovery from his illness. This ...

Irritable bowel syndrome is a functional gastrointestinal illness, defined by symptoms. Irritable bowel syndrome has been described as a biopsychosocial condition, in which colonic dysfunction is affected by psychological and social factors. As a result of this unusual constellation, irritable bowel syndrome may be subject to cultural variables that differ ...

In the last 3 decades, there has been a growing appreciation for the role of gut bacteria in gastrointestinal disease. Although inflammatory bowel disease and peptic ulcer disease have established a role for gut bacteria for more than 20 years, there is now increasing evidence that intestinal bacteria may play ...

Numerous clinical subtypes of Guillain-Barré syndrome have been described over the century since the original description of the syndrome. These variants of Guillain-Barré syndrome are discussed and their immunological pathogenesis reviewed.

Guillain-Barre syndrome (GBS) is the rubric encompassing highly variable phenotypic subgroups of acute, postinfectious, immune-mediated peripheral neuropathy. The hallmark of GBS phenomenology is a rapidly progressive ascending lower extremity weakness. GBS taxonomy includes a motor and sensory axonal neuropathy (AMSAN). Nitrous oxide (NO) abuse may create a pattern of neurological ...

Importance of the field: Irritable bowel syndrome (IBS) represents one of the most common gastrointestinal disorders, with the diarrhea-predominant form (D-IBS) representing an area of high unmet medical need. One difficulty in identifying suitable treatments for IBS is that although there are animal models for the components of IBS, it ...

The authors describe the demographics, clinical presentation, investigation, treatment, and outcomes of pediatric patients with Guillain-Barré syndrome. They identified 35 pediatric patients with Guillain-Barré syndrome presenting to a tertiary academic center over a 20-year period. The most common presenting symptoms were paresthesias (54%), weakness (49%), and myalgias (49%). Sensation was ...

Dyspepsia is a highly prevalent condition characterized by symptoms originating in the gastroduodenal region without underlying organic disorder. Treatment modalities include acid-suppressive drugs, gastroprokinetic drugs, Helicobacter pylori eradication therapy, tricyclic antidepressants, and psychological therapies. Irritable bowel syndrome is a multifactorial, lower functional gastrointestinal disorder involving disturbances of the brain-gut axis. ...

Authors describe a patient who developed a myelopathy associated with Guillain-Barré syndrome and cervical myelopathy. We provide radiological evidence of non-compressive herniated cervical intervertebral disc with cord signal changes and show the clinical and electrophysiological result of coexisting Guillain-Barré syndrome and cervical myelopathy. We tried to introduce and review the ...

OBJECTIVE: To use the International Classification of Functioning, Disability and Health (ICF) to describe and compare patient-reported disability in Guillain-Barré syndrome survivors and persons with multiple sclerosis, and to identify relevant environmental factors. METHODS: Cross-sectional survey of 77 survivors of Guillain-Barré syndrome in the community. Their Guillain-Barré syndrome-related problems were ...

Guillain-Barré syndrome is an autoimmune disease which is a common cause of acute neuromuscular paralysis and should be suspected in all patients with unexplained motor weakness. Specific management strategies are more effective if given early in the course of the disease.

The author reviews current treatment approaches for the management of acute immune-mediated peripheral neuropathies, including the Guillain-Barré syndrome together with the clinical variants, which are acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and the Fisher syndrome. A summary of clinical evidence for drug ...

Irritable bowel syndrome (IBS) is a multifactorial disease during which the pathophysiological role of the gut microbiota has been recently highlighted. In almost 20% of the patients, IBS is clearly a post-infectious IBS as a consequence of an acute bacterial gastroenteritis. Some papers have reported an abnormal colonic fermentation in ...

Questions from patients about analgesic pharmacotherapy and responses from the authors are presented to help educate patient sand make them more effective self-advocates. The topics addressed in this issue are the signs, symptoms, and treatment of irritable bowel syndrome (IBS). A discussion of pain management and complementary therapies is included.

Cerebral salt-wasting syndrome and the syndrome of inappropriate antidiuresis (SIAD) are the most important causes of non-iatrogenic hyponatraemia that can significantly complicate various brain diseases. Salt wasting without an underlying CNS disease may have been disregarded so far by clinicians and has been described as renal salt-wasting (RSW) in patients ...