mycophenolic-acid and Deglutition-Disorders

To describe a case of coincident Castleman's disease and myasthenia gravis that initially presented as rapidly progressive dysphagia and dysphonia and to review the unique pathophysiology of these two uncommon diagnoses.. Case report and literature review.. Castleman's disease, angiofollicular or giant lymph node hyperplasia, is a rare benign lymphoid proliferation. Traditionally, the disease is classified based on histologic and clinical characteristics. Fewer than 10 cases with concurrent myasthenia gravis have been reported. Myasthenia gravis and thymic epithelial tumors are both associated with acetylcholine receptor antibody. While patients with isolated Castleman's disease are usually asymptomatic, those who have concurrent myasthenia gravis and undergo surgical treatment are at increased risk of postoperative myasthenic crisis. Both pre- and postoperative plasmapheresis are suggested to improve muscle strength and prevent severe postoperative complications.. In the setting of multiple cranial neuropathies including velopalatal insufficiency and bilateral ptosis it is important to consider myasthenia gravis. Castleman's disease occurs rarely in conjunction with myasthenia gravis but may increase the risk of myasthenic crisis.

Topics: Adolescent; Castleman Disease; Cholinesterase Inhibitors; Deglutition Disorders; Dysphonia; Female; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Myasthenia Gravis; Mycophenolic Acid; Prednisone; Pyridostigmine Bromide; Thymectomy; Tomography, X-Ray Computed; Velopharyngeal Insufficiency

Pemphigus vulgaris (PV) is a rare autoimmune bullous disease which affects the skin and mucous membranes. Oesophageal involvement is rare and has previously been limited to case reports and case series. A recent large case series of 477 PV patients showed that 26/477 (5.4%) had symptomatic oesophageal involvement. We present the case of a 54-year-old Somalian lady with a 10-year history of cutaneous PV, currently in remission, who developed dysphagia and odynophagia and was subsequently found to have oesophageal PV involvement with multiple flaccid bullae which were positive for anti-DSG3 antibodies on in-direct immunofluorescence. She had her treatment switched from azathioprine to mycophenolate and prednisolone, leading to resolution of her symptoms.

Topics: Anti-Inflammatory Agents; Autoantibodies; Deglutition Disorders; Desmoglein 3; Esophageal Diseases; Female; Humans; Immunologic Factors; Microscopy, Fluorescence; Middle Aged; Mycophenolic Acid; Pain; Pemphigus; Prednisolone

The number of contrast media-related procedures is ever increasing due to the widespread availability of theoretically safe, low osmolar iodinated contrast material. Although intravenously administered contrast is known to precipitate myasthenic crisis, oral contrast aspiration as a causative factor is not yet documented as such. A 48-year-old Sinhalese man diagnosed as having myasthenia gravis, was evaluated for progressive dysphagia with an upper gastrointestinal contrast study. Iodinated contrast material (iohexol) was used as the contrast medium and there was direct evidence of contrast aspiration during the study. Several minutes after the procedure, severe respiratory distress with evidence of myasthenic crisis requiring intubation and intensive care admission was noted. Treatment with intravenous immunoglobulin, high-dose steroids, and broad-spectrum intravenously administered antibiotics led to an uneventful recovery, although the latter part of the clinical course was complicated with total left lung collapse. Myasthenic crisis can be precipitated by various factors and a successful recovery requires mechanical respiratory support with immunomodulatory and steroid therapy. This is the first reported case that describes the development of myasthenic crisis following iohexol-associated aspiration pneumonitis.

Topics: Cholinesterase Inhibitors; Contrast Media; Deglutition Disorders; Disease Progression; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Immunosuppressive Agents; Iohexol; Male; Middle Aged; Myasthenia Gravis; Mycophenolic Acid; Pneumonia, Aspiration; Pneumothorax; Prednisolone; Pyridostigmine Bromide; Respiration, Artificial; Respiratory Aspiration; Respiratory Insufficiency

To report the use of intravenous immunoglobulin (IVIg) and mycophenolate mofetil (MMF) in polymyositis (PM) and dermatomyositis (DM).. We performed an open study in PM and DM with active disease. Indications for treatment were: steroid-dependency, refractoriness to steroid and/or immunosuppressants, and life-threatening disease. IVIg was used at 2 g/kg in monthly cycles for six months and then each other month for other three cycles. MMF was slowly titrated to 30 mg/kg/day orally. Parameters employed to follow patients were the Medical Research Council (MRC) scale, the modified Rankin score, CK serum levels and daily prednisone dose.. Seven patients were studied (4PM, 3DM). All were females, with a mean age of 49 years. All of them achieved a complete remission and, at the last follow-up visit, significant differences in MRC score, modified Rankin score, CK levels, and the daily maintenance prednisone dose were documented. No relevant side effects were observed.. IVIg as add on treatment with MMF is effective in severe and refractory myositis, moreover as safe and steroid-sparing agent.

Topics: Aged; Creatine Kinase; Deglutition Disorders; Dermatomyositis; Disease Progression; Drug Resistance; Drug Therapy, Combination; Dyspnea; Female; Follow-Up Studies; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Middle Aged; Mycophenolic Acid; Polymyositis; Remission Induction