mycophenolic-acid and Muscle-Weakness

Topics: Antirheumatic Agents; Autoantibodies; Child, Preschool; Cyclophosphamide; Dermatomyositis; Diagnosis, Differential; Glucocorticoids; Humans; Male; Muscle Weakness; Mycophenolic Acid; Penis; Scrotum; Ulcer

Immune-mediated necrotising myopathy (IMNM) is a type of inflammatory myopathy characterised by acute or subacute severe proximal muscle weakness, significantly elevated creatine kinase levels, and prominent myofibre necrosis and regeneration with little or no inflammation. A subtype of IMNM identified by anti-HMG-CoA reductase (HMGCR)antibodies has been shown to be associated with statin exposure. Treatment of IMNM consists of immunosuppression with steroids, steroid-sparing agents, intravenous immune globulin and/or biologics. We present here a case of anti-HMCGR-associated IMNM and review the pathophysiology, diagnosis and treatment to increase physician awareness of this rare and debilitating condition.

Topics: Administration, Intravenous; Aged; Autoimmune Diseases; Biopsy; Creatine Kinase; Diagnosis, Differential; Enzyme Inhibitors; Glucocorticoids; Humans; Hydroxymethylglutaryl CoA Reductases; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Immunoglobulins, Intravenous; Male; Methylprednisolone; Muscle Weakness; Muscular Diseases; Mycophenolic Acid; Myositis; Necrosis; Treatment Outcome

Topics: Antihypertensive Agents; Cyclophosphamide; Cyclosporine; Drug Substitution; Drug Therapy, Combination; Fatigue; Glomerulosclerosis, Focal Segmental; Gynecomastia; Humans; Hypertension; Male; Mastodynia; Middle Aged; Muscle Weakness; Mycophenolic Acid; Prednisone; Proteinuria; Tacrolimus

Dermatomyositis is an inflammatory myopathy and commonly presents with progressive, symmetric proximal muscle weakness and cutaneous findings. Calcinosis is a severe manifestation that can be debilitating. The cutaneous manifestations of dermatomyositis may also develop in the absence of detectable muscle disease, and can persist after the successful treatment of myositis. The author reports a 30-year-old woman with biopsy-proven dermatomyositis who had failed previous trials of azathioprine and methotrexate. Her muscle weakness was controlled with mycophenolate mofetil and prednisone; however, she had recurrent attacks of painful calcinosis. The patient responded to intravenous immune globulin (IVIG) along with intravenous methylprednisone, followed by IVIG for 2 consecutive days each month. This regimen has been effective in preventing recurrence of her calcinosis. This case illustrates the cutaneous manifestation of dermatomyositis, which is often more refractory to treat as compared to the muscle involvement and require additional approaches such as IVIG.

Topics: Adult; Anti-Inflammatory Agents; Calcinosis; Dermatomyositis; Female; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Immunosuppressive Agents; Methylprednisolone; Muscle Weakness; Mycophenolic Acid; Prednisone; Secondary Prevention

The term "trabecular myopathy" has been used to designate a syndrome resembling limb-girdle muscular dystrophy in which the predominant pathological feature is an abundance of lobulated or trabecular muscle fibers. However, the validity of this nosological entity has not been verified. Herein we describe a 63-year-old man with a severe, progressive myopathy who exhibited the typical pathological features of both trabecular myopathy and nemaline myopathy in association with a biclonal gammopathy. In this case, adult-onset nemaline myopathy was probably the primary disease process. The diagnostic significance of trabecular muscle fibers remains uncertain.

Topics: Biopsy; Disease Progression; Humans; Immunosuppressive Agents; Inclusion Bodies; Male; Middle Aged; Muscle Fibers, Skeletal; Muscle Weakness; Muscle, Skeletal; Mycophenolic Acid; Myopathies, Nemaline; Paraproteinemias; Prednisone

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may occur in association with diabetes mellitus (DM). We report a case of a poorly controlled diabetic patient who presented with rapid onset of bilateral lower extremity weakness and sensory loss associated with sacral and posterior thigh paresthesias and urinary and bowel incontinence, indicative of cauda equina syndrome (CES). Subsequent evaluation was consistent with CIDP. Monthly infusions with intravenous immunoglobulins (IVIg) with strict glycemic control using insulin resulted in remarkable clinical and electrophysiological recovery. This case report describes a rare presentation of CIDP and emphasizes the importance of early utility of electrodiagnostic (EDX) studies in the clinical evaluation of diabetic patients presenting with rapidly progressive lower extremity weakness and sensory loss associated with diminished reflexes.

Topics: Aged; Central Nervous System; Diabetic Neuropathies; Diagnosis, Differential; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Muscle Weakness; Mycophenolic Acid; Paresis; Peripheral Nerves; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Polyradiculopathy; Somatosensory Disorders; Treatment Outcome; Urination Disorders

Topics: Adult; Anti-Inflammatory Agents; Anti-Inflammatory Agents, Non-Steroidal; Autoantibodies; Brain; Cholinesterase Inhibitors; Electroencephalography; Epilepsy; Female; Humans; Immunoglobulins, Intravenous; Memory Disorders; Muscle Weakness; Muscle, Skeletal; Myasthenia Gravis; Mycophenolic Acid; Prednisone; Pyridostigmine Bromide; Receptor Protein-Tyrosine Kinases; Receptors, Cholinergic; Treatment Outcome

Mycophenolate mofetil (MMF) is a new immunosuppressive agent currently being used for the prevention of renal allograft rejection. MMF is a specific inhibitor of lymphocytes and is well tolerated leading to its use in other autoimmune diseases. We have used MMF for the treatment of seven patients with inflammatory myopathy and are hereby reporting our results.. All of our patients were females (age 17-65 yr). They were symptomatic upon presentation and met classification criteria for idiopathic inflammatory myopathy. Inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein as well as creatine kinase were significantly elevated in all the patients, indicating active disease. Corticosteroids were concomitantly being administered (20-60 mg/day of prednisone). Initial therapy with conventional immunosuppressives was either ineffective or had significant adverse effects leading to their discontinuation. MMF was started in doses of 500 mg twice a day and titrated up to 1 g twice a day.. Our patients have exhibited an impressive serological response to therapy with MMF and six patients had a marked improvement in their weakness. One patient had incomplete improvement in her weakness and has required additional therapies. MMF has been well tolerated during the treatment period (12-36 months).. A striking clinical and laboratory response of active myositis in six out of seven patients in this series illustrates that MMF can be effectively used in management of autoimmune inflammatory myopathy and may be a suitable alternative to the conventional immunosuppressive agents.

Topics: Adolescent; Adult; Aged; Autoimmune Diseases; Dermatomyositis; Female; Humans; Immunosuppressive Agents; Middle Aged; Muscle Weakness; Mycophenolic Acid; Myositis; Polymyositis; Treatment Outcome