Guillain–Barre in a patient with ankylosing spondylitis secondary to ulcerative colitis on infliximab therapy

We describe a 47-year-old woman with severe spondylarthropathy secondary to ulcerative colitis who developed a Guillain–Barre after the use of anti-TNF-α. She first developed ulcerative colitis in November 1997. In 2003, she developed uveitis and, in 2005, axial and enthesitis form of spondylarthropathy. In May 2007, her condition was exacerbated. Therapy with infliximab has been initiated. The patient received 5-mg/kg infusions of infliximab. She had significant improvement in her arthritis and was in remission for her ulcerative colitis. She was admitted to the hospital 2 weeks after her third dose of infliximab for having developed paraesthesia of her hands and lower limbs. Neurophysiology studies demonstrated an acquired segmental demyelinating polyneuropathy consistent with Guillain–Barre syndrome (GBS). Laboratory investigations were unremarkable. She was treated with intravenous corticosteroids with no improvement. After this, she received infusions of intravenous gammaglobulin (IVIg) with complete recovery of the muscle strength within a few weeks. A follow-up electromyographic study 3 months later showed normal finding. The development of GBS in our patient may be secondary to her anti-TNF-α treatment. At present, she remains off anti-TNF-α therapy.     

The association between dermatomyositis and papillary thyroid cancer: a case report

We report the case of a 66-year-old woman who developed progressive proximal muscle weakness and papillary thyroid cancer. After a thyroidectomy, she was treated with intravenous methylprednisolone pulse therapy and oral prednisolone followed by tacrolimus therapy. However, her clinical symptoms and laboratory data did not improve sufficiently. Therefore, we administered intravenous immunoglobulin. As a result, she regained substantial muscle strength along with complete normalization of serum muscle enzymes and showed no evidence of recurrence of papillary thyroid cancer or exacerbation of dermatomyositis (DM). Although there is controversy as to whether papillary thyroid cancer is involved in DM, the results of this study support a connection between these two conditions.     

Successful treatment of acquired hemophilia A, complicated by chronic GVHD, with tocilizumab

A 65-year-old woman who had suffered from chronic graft-versus-host disease (GVHD) presented with extensive purpura and was diagnosed with acquired hemophilia A. Because she was refractory to corticosteroids and her condition was complicated with diabetes mellitus, glaucoma, and hypoglobulinemia, she was treated with tocilizumab. Tocilizumab treatment increased the activity of factor VIII in a rapid and sustained manner, leading to a reduction of the prednisolone dose. Tocilizumab may thus be an optional treatment modality for acquired hemophilia A.     

Treatment of inclusion body myositis: is low-dose intravenous immunoglobulin the solution?

Inclusion body myositis (IBM), the most common inflammatory myopathy in the elderly, is often resistant to various forms of therapy. Placebo-controlled treatment trials with high dose intravenous immunoglobulins (IVIG) have shown disease amelioration in some but not all patients. Here, we present the informative case of a 70-year-old woman with diagnosed inclusion body myositis that showed progressive muscle weakness without treatment and following immuno-suppressive treatment with corticosteroids and azathioprine. A trial with low-dose intravenous immunoglobulins was started at that time. The patient responded rapidly to low dose IVIG treatment with amelioration of muscle strength and normalization of CK serum activities. Our results demonstrate that IBM patients may respond to low-dose IVIG treatment which has important clinical and economic consequences.     

Malignant lymphoma originating in the cauda equina mimicking the inflammatory polyradiculoneuropathy

A 67-year-old woman was diagnosed with inflammatory polyradiculoneuropathy. The intravenously administered immunoglobulin (IVIG) treatment that she received several times over a 3-year period relieved her clinical symptoms of muscle weakness and sensory disturbances, but these symptoms had worsened thereafter despite further IVIG treatment. MRI detected a solid tumor involving the cauda equina and pathological examinations confirmed this to be malignant lymphoma. The clinical and radiological findings for the malignant lymphoma of the cauda equina in this patient were quite similar to those for the inflammatory polyradiculoneuropathy.     

Rapidly progressive interstitial lung disease in a dermatomyositis patient with high levels of creatine phosphokinase,severe muscle symptoms and positive anti-Jo-1 antibody

It has been reported that there is a subgroup of dermatomyositis (DM) patients with rapidly progressive interstitial lung disease (ILD) who have mild muscle symptoms, slightly increased levels of muscle enzymes, and absence of anti-Jo-1 antibody. A 51-year-old woman with DM was intubated requiring mechanical ventilation because of a rapidly progressing ILD in spite of the absence of the typical poor prognostic factors. A high dose or pulse therapy of corticosteroids was not effective, but additional treatment of cyclosporine gradually improved her respiratory condition. It is not clear why a rapidly progressive ILD occurred in this case lacking poor prognostic factors. However, if corticosteroid treatment is not effective, additional administration of cyclosporine in the early period of rapidly progressive ILD may rescue deteriorating cases.     

Dermatomyositis with splenic and renal infarctions during corticosteroid therapy

A 60-year-old woman was admitted to our hospital with complaints of muscle weakness and erythema on her extremities. Gottron's sign, heliotrope rash, elevation of serum myogenic enzymes, electromyography and magnetic resonance imaging findings established a diagnosis of dermatomyositis (DM). She was treated with 60 mg of daily prednisolone. One week later, she suddenly developed splenic and renal infarctions, which were considered to have resulted from vasculopathy associated with DM. Cyclophosphamide and anticoagulants along with increasing the dosage of corticosteroid were effective. This is the first report describing splenic and renal infarctions in a patient with adult-onset DM.     

Successful treatment of psychosis secondary to SLE with high dose intravenous immunoglobulin.

A 23-year-old woman with SLE was admitted because of severe psychosis manifested by depression, delusions and the inability to perform minimal daily activities. The patient refused treatment with steroids, but was later convinced to try treatment with intravenous immunoglobulin (IVIG). Following treatment with IVIG a marked improvement was noted in her mental status and she was discharged. During a follow-up period of 18 months she resumed normal life; she does not receive any drugs currently and no psychiatric abnormalities have been noted. It is suggested that IVIG may be considered in the treatment of lupus cerebritis, especially when serious complications develop and other treatment modalities are ineffective.     

Prospective study of high-dose intravenous immunoglobulin for the treatment of steroid-resistant polymyositis and dermatomyositis

Abstract

High-dose intravenous immunoglobulin (IVIG) therapy has been effective in treating many autoimmune and systemic inflammatory diseases. In the present prospective study, we evaluated the efficacy of IVIG for patients with polymyositis (PM) and dermatomyositis (DM) refractory to treatment with high-dose corticosteroids. PM/DM was defined as steroid-resistant when the muscle strength of a patient did not improve despite the administration of more than 50?mg prednisolone per day for more than 4 weeks. A total of 12 patients with biopsy-proven, steroid-resistant PM/DM received one infusion of polyethylene glycol-treated human IgG at a dose of 0.4?g per kg per day for five successive days. Three of the patients received a second infusion. All patients were followed for up to 3 months after the infusion. Finally, 8 patients (6 PM and 2 DM; 5 men and 3 women) aged 29–67 years (mean 48 years) were analyzed. Their clinical response was assessed by changes in (a) subjective signs, i.e., fatigue (visual analog scale, VAS), muscle pain (VAS), activities of daily living (ADL), (b) objective signs, i.e., manual muscle strength (MMT) and serum level of creatine kinase (CK). At 12 weeks after the infusion, the patients showed significant improvement in their scores of muscle strength (from a mean of 67.0 to 81.0) and their ADL scores (from a mean of 27.1 to 39.1). The mean serum CK level decreased significantly from 1287.4 to 612.6?IU/l. In addition, the mean VAS of fatigue decreased significantly from 5.5 to 1.3?cm. The physicians’ assessment showed that 87.5% of patients had improved. The average reduced dose of prednisolone was 47.1?mg/day at 12 weeks after infusion in 7 patients who exhibited improvement. Adverse effects, i.e., asymptomatic myocardial infarction and increased blood urea nitrogen (BUN), were noted with two of the 15 infusion (13%). Overall, IVIG was found to be safe and effective for refractory PM and DM.     

Intravenous immunoglobulin therapy in adult patients with polymyositis/dermatomyositis: a systematic literature review

The objectives of this study are to review and summarize published information on the use, effectiveness, and adverse effects of intravenous immunoglobulin (IVIG) in patients with polymyositis (PM) or dermatomyositis (DM) and to search MEDLINE and CNKI (Chinese) databases from 1985 to 2011 to retrieve clinical research articles concerning IVIG in adult patients with PM/DM. Of the 14 articles selected, two were randomized controlled trials, nine prospective open studies, and three retrospective studies with a total of 308 adult patients. IVIG has been used successfully in the treatment of PM/DM. The standard dose is 2 g/kg, given in two to five individual daily doses. The course of IVIG treatment is usually 3~6 months. IVIG therapy seemed rarely employed as first-line therapy in PM/DM. In a double-blind study conducted in patients with refractory DM, IVIG combined with corticosteroid significantly improved muscle strength and decreased serum creatine kinase level, compared with placebo. The beneficial effect of IVIG in refractory, flare-up, rapidly progressive, or severe PM/DM has been documented in many open-label trials. IVIG was shown to be effective in most of PM/DM patients with lung involvement and esophageal involvement. In some patients, IVIG can lower the corticosteroid dose required for maintenance, demonstrating the most effective steroid-sparing effect. Adverse effects were generally tolerable. IVIG is effective in the treatment of adult patients with PM/DM and appears to be relatively well tolerated and safe. IVIG may be a good choice especially in patients with refractory, flare-up, rapidly progressive, or severe PM/DM, and can be tried in patients with a contraindication for corticosteroid.     

Successful treatment of new onset Wegener’s granulomatosis with IVIG (intravenous immunoglobulin) during pregnancy: a case report

We describe a patient with limited Wegener’s granulomatosis (WG) presenting during pregnancy with aggressive cutaneous involvement. She was treated with a combination of high-dose corticosteroids and intravenous immunoglobulin (IVIG) during her third trimester. The patient had otherwise uneventful pregnancy and a satisfactory outcome for both herself and her newborn. In the English literature, prior to this report, there have been de novo cases of WG in pregnant women that were diagnosed and treated during pregnancy and three cases of WG treated successfully with IVIG during pregnancy.     

Rituximab for the treatment of juvenile dermatomyositis: a report of four pediatric patients

OBJECTIVE: Juvenile dermatomyositis (DM) is a chronic inflammatory myopathy of childhood primarily affecting the muscles and skin. Treatment for juvenile DM is often difficult, and conventional therapies include corticosteroids and other immune suppressants. We reviewed the records of 4 patients with juvenile DM who received the B cell-depleting anti-CD20 monoclonal antibody rituximab to determine whether this therapy resulted in improved control of their juvenile DM. METHODS: This is a retrospective review of 4 pediatric patients ages 10-17 years with juvenile DM who were treated with rituximab. All patients were tested for myositis autoantibodies and received weekly rituximab infusions for a total of 4 doses. Two patients received repeat courses of rituximab 1 year after their first dose. Patients were followed up between 12 and 24 months after their first course of rituximab, and their strength, muscle enzymes, and rash were reviewed. RESULTS: One patient was positive for a myositis-specific antibody, anti-Mi-2, and demonstrated striking reductions in her muscle enzyme levels for 1 year after rituximab therapy. Following a second course of rituximab, this patient remained disease free for 14 months before requiring a third course of rituximab. Two myositis antibody-negative patients showed clinical improvement and tolerated lower doses of corticosteroids following treatment with rituximab. Finally, 1 patient had worsening of her disease following rituximab. CONCLUSION: These cases highlight the potential for anti-B cell therapies in the treatment of juvenile DM in both myositis-specific autoantibody-positive and -negative patients.     

A case of dermatomyositis with “liver disease associated with rheumatoid diseases” positive for anti-liver–kidney microsome-1 antibody

We reported a case of dermatomyositis (DM) with liver disturbance in a 50-year-old Japanese female. She presented with fever, muscle weakness, and typical DM rashes. On clinical and serological examinations, the liver impairment was initially diagnosed as probable autoimmune hepatitis, which was denied by a histological study despite positive anti-liver–kidney microsome-1 antibody. Finally, she was diagnosed as having DM with “liver disease associated with rheumatoid diseases”, and treatment with oral prednisone (40 mg/day) achieved normalization of liver and muscle enzyme levels as well as improvement of symptoms associated with DM. Liver involvement in patients with polymyositis (PM)/DM has not been well described and is considered to be uncommon. Full clarification of the etiology of liver impairment with a histological examination in collagen diseases including PM/DM is useful to determine the proper dose of corticosteroids for the treatment of collagen diseases and their liver complications.     

Sequential Plasmapheresis and Intravenous Immunoglobulin Therapy for Refractory Myasthenia Gravis: Case Report

Immunotherapy is currently the standard therapy for myasthenia gravis (MG) although some patients may be refractory to treatment. We describe the use of sequential plasmapheresis and intravenous immunoglobulin (IVIG) therapy for treatment of advanced MG in a patient refractory to all forms of medical treatment including corticosteroids, immunosuppressants, and intermittent plasmapheresis. The patient, a 37-year-old woman with systemic lupus erythematosus (SLE), had initially responded well to treatment with high dose corticosteroids and intermittent plasmapheresis, with the duration of response ranging from 3 to 4 months. However, after 18 months of therapy, the duration of response had gradually decreased to 1 month. She responded well to a 5 day trial of plasmapheresis followed by high dose IVIG, and the duration of response increased to 6 months. The SLE activity was relatively silent during each relapse. This report indicates the potential usefulness of sequential plasmapheresis and IVIG in the treatment of patients with refractory MG and SLE.     

Inflammatory Myopathy Associated with Anti-mitochondrial Antibody Presenting Only with Respiratory Failure

A 56-year-old woman presenting with type II respiratory failure was transferred to our hospital. She did not exhibit muscle weakness or elevated serum myogenic enzymes, but needle electromyography revealed myogenic changes in the limb muscles, and her blood tests were positive for anti-mitochondrial antibodies (AMA). Muscle histopathological findings included immune-mediated necrotizing myopathy, so she was diagnosed with inflammatory myopathy associated with AMA. After treatment with corticosteroids and noninvasive positive pressure ventilation, her symptoms improved. If a diagnosis of type II respiratory failure is difficult, inflammatory myopathy associated with AMA should be considered as a differential diagnosis.     

The Efficacy of Therapeutic Plasmapheresis for the Treatment of Fatal Hemophagocytic Syndrome: Two Case Reports

Abstract: A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppresants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosupressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral DNA from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).     

Churg-Strauss syndrome with myocarditis manifesting as acute myocardial infarction with cardiogenic shock: case report and review of the literature

A patient with a two-year history of worsening asthma presented with chest pain and shortness of breath. She developed cardiogenic shock. Analysis of blood chemistry detected increased troponin I concentration. Her electrocardiographic changes were consistent with a diagnosis of anteroseptal myocardial infarction. However, angiography showed normal coronary arteries. Left ventriculography showed severe mitral regurgitation and global hypokinesis. Peripheral eosinophilia was detected. Subsequent endomyocardial biopsy showed myocarditis with prominent eosinophil and plasma cell components. Churg-Strauss syndrome was diagnosed based on her history of asthma, evidence of peripheral eosinophilia and results of endomycardial biopsy. Treatment with a high dose of corticosteroids was initiated. As symptoms of heart failure improved - without recurrence of cardiac and respiratory symptoms - the dose of corticosteroids was gradually reduced. Eight months after her original presentation, she developed urticarial lesions on her abdomen and legs, with muscle soreness but no other associated symptoms. She was treated with a combination of prednisone and dapsone. After the diagnosis of Churg-Strauss syndrome, the patient remained symptom free with a normal ejection fraction for 15 months while taking prednisone.     

Oculomotor muscles involvement revealing dermatomyositis in a patient with rheumatoid arthritis

INTRODUCTION: Oculomotor muscles (OMM) involvement in dermatomyositis (DM) and in rheumatoid arthritis (RA) is unusual. The DM always leads to OMM inflammation, whereas the RA particularly leads to tenosynovitis of the superior oblique muscle referred to as the Brown syndrome. OBSERVATION: The patient is a 43-year-old woman who gives a 17-year-history of severe seropositive RA with bilateral coxite. She was hospitalized for acute painful proptosis. The clinical examination revealed an orbital erythema and a muscular rhizomelic weakness. The muscular enzymes were increased. The orbital CT revealed in the right side, an enlargement of the superior rectus muscle that was enhanced after intravenous injection, which is compatible with myositis involvement. The muscular biopsy practiced at the level of the calf showed the specific histological signs of the DM. This orbital involvement was resolved with a high dose of corticosteroids. CONCLUSION: Our observation has the specificity of associating RA with DM with an involvement of the superior rectus muscle, which is due to the DM rather than the RA.     

Limited effects of high-dose intravenous immunoglobulin (IVIG) treatment on molecular expression in muscle tissue of patients with inflammatory myopathies

OBJECTIVES: The study was conducted with the aim of achieving an improved understanding of the molecular mechanisms of high-dose intravenous immunoglobulin (IVIG) in inflammatory myopathies by investigating the effects on muscle function and immunological molecules in skeletal muscle of polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM) patients. METHODS: Thirteen treatment-resistant patients, 6 PM, 4 DM, 2 IBM and 1 juvenile DM, were treated with 2 g/kg of IVIG, three times at monthly intervals. Functional Index in Myositis and serum creatinine kinase (CK) levels were determined, and muscle biopsies were performed before treatment and after the third IVIG infusion. Immunological molecules were also studied in biopsies taken 24-48 h after the first infusion. RESULTS: Improved muscle function was observed in three patients (1 PM, 1 DM and 1 IBM) and CK levels decreased in five. T cells, macrophages, major histocompatibility complex (MHC) class I antigen on muscle fibres, intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) expression and membranolytic attack complex (MAC) deposits on capillaries were present to an equal degree in biopsies before and after IVIG treatment. No correlation between the clinical response and molecular changes was found. CONCLUSIONS: The clinical effects of high-dose IVIG on muscle function in patients with refractory inflammatory active myositis did not correspond to effects on any of the investigated molecules in our study. T cells, macrophages, phenotypical changes in muscle fibres and endothelial cell activation were still present after treatment. These observations question a role for IVIG as an immune-modulating therapy in patients with inflammatory myopathies.     

Liver cirrhosis as a delayed complication of Stevens-Johnson syndrome

A 26-year-old woman was referred to our department due to fever and skin rash after having taken medication for a common cold. Physical examination revealed erythematous skin changes on her body associated with mucosal involvement in her eyes and oral cavity. Peripheral blood examination revealed leukopenia and thrombocytopenia. Liver function test showed hyperbilirubinemia. She was managed with high dose intravenous immunoglobulin (IVIG) at 1.0 gm/kg of body weight infused for 5 consecutive days. Although the patient's skin lesion improved dramatically with IVIG therapy, her hyperbilirubinemia aggravated progressively. Eighteen months after her presentation, liver cirrhosis was diagnosed by ultrasonography, laboratory and liver biopsy findings.