Although corticosteroids have been the initial agent for the treatment of inflammatory myopathies (IM), immunosuppressive agents such as azathioprine, methotrexate, cyclophosphamide, or cyclosporine are commonly required to control the disease except mild cases. On the other hand, the efficacy of combination therapy of cyclosporine and methotrexate in severe rheumatoid arthritis has been proven without serious side effects. However, in treatment-resistant myositis, the experience of such a therapy is very limited, and has not been described in refractory polymyositis with anti-Jo-1 antibody. Here, we report a young female patient with recalcitrant polymyositis and anti-Jo-1 antibody who was successfully treated with the combination therapy of cyclosporine and methotrexate. At first, the myositis did not respond to several agents, such as corticosteroid, monthly pulse cyclophosphamide, azathioprine, or cyclosporine. Methotrexate was initially avoided as treatment regimen because of its potential pulmonary toxicity in the case with preexisting lung disease.
Adult ; Antibodies, Antinuclear/blood* ; Autoantigens/immunology ; Cyclosporine/administration & dosage ; Cyclosporine/therapeutic use* ; Drug Resistance ; Drug Therapy, Combination ; Female ; Histidine-tRNA Ligase/immunology ; Human ; Immunosuppressive Agents/administration & dosage ; Immunosuppressive Agents/therapeutic use* ; Methotrexate/administration & dosage ; Methotrexate/therapeutic use* ; Polymyositis/drug therapy* ; Polymyositis/immunology

Bucillamine is a disease modifying anti-rheumatic drug, structurally similar to D-penicillamine. Although D-penicillamine-induced pemphigus has been not infrequently demonstrated, pemphigus associated with bucillamine was rarely reported. We describe a patient complicating pemphigus vulgaris after bucillamine treatment in rheumatoid arthritis (RA) and polymyositis (PM) overlap syndrome. PM and RA overlap syndrome was diagnosed three years ago and bucillamine was administrated for 20 months. Skin lesions including erythematous flaccid blisters on her chest, axillae, and back were occurred and were compatible with pemphigus vulgaris by typical pathology. Withdrawal from bucillamine and prednisolone treatment made rapid improvement of pemphigus lesions.
Syndrome ; Skin/pathology ; Polymyositis/*complications/*drug therapy ; Pemphigus/*chemically induced/*pathology ; Middle Aged ; Humans ; Female ; Cysteine/adverse effects/*analogs & derivatives ; Biopsy ; Arthritis, Rheumatoid/*complications/*drug therapy ; Arthritis ; Antioxidants/*adverse effects

Polymyositis is a rare complication of interferon alpha treatment as a result of immunemodulating role of the drug itself. In this case, interferon alpha induced polymyositis and cardiomyopathy is diagnosed in a 33-yr-old male patient with history of chronic hepatitis B. To treat hepatitis B, interferon alpha was administered until the proximal muscle weakness developed. Thereafter, sixteen cycles of immunoglobulin treatment (400 mg/kg) along with corticosteroids were instituted and led to an improvement in subjective symptoms with decreases in level of CPK and LDH. However, dilated cardiomyopathy has not improved in spite of the cessation of interferon treatment. Unlike the persistently elevated serum HBV DNA level, the serum ALT and AST levels have gradually decreased. Our case shows that clinical symptoms of polymyositis improved with steroid and immunoglobulin treatment without deterioration of the hepatitis B. To our knowledge, this is the first case of polymyositis associated with dilated cardiomyopathy after the administration of interferon in a patient with hepatitis B.
Adrenal Cortex Hormones/therapeutic use ; Adult ; Antigens, CD13/blood ; Antiviral Agents/*adverse effects/therapeutic use ; Aspartate Aminotransferases/blood ; Cardiomyopathy, Dilated/blood/*chemically induced/drug therapy/physiopathology ; Hepatitis B, Chronic/blood/complications/*drug therapy ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Interferon-alpha/*adverse effects/therapeutic use ; Male ; Polymyositis/blood/*chemically induced/drug therapy/physiopathology ; Treatment Outcome

Pneumatosis cystoides intestinalis (PCI), characterized by presence of intramural gas cyst in the intestinal wall is associated with various medical condition. Polymyosistis, however, is rarely associated with PCI. Few cases are reported in the world, and none has not been reported previously in Korea. A 67-year-old woman with polymyositis developed mild abdominal pain and abdominal distension during treatment with steroid and azathioprine. Radiographic findings including CT scan showed intraperitoneal free gas and intramural air, compatible with PCI. The patient's symptom and clinical findings improved after the treatment with antibiotics and high-dose oxygen therapy.
Aged ; Anti-Bacterial Agents/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Azathioprine/therapeutic use ; Cefotaxime/therapeutic use ; Female ; Humans ; Oxygen Inhalation Therapy ; Pneumatosis Cystoides Intestinalis/complications/*diagnosis/drug therapy ; Polymyositis/complications/*diagnosis/drug therapy ; Prednisolone/therapeutic use ; Radiography, Abdominal ; Tomography, X-Ray Computed

Pneumatosis cystoides intestinalis (PCI), characterized by presence of intramural gas cyst in the intestinal wall is associated with various medical condition. Polymyosistis, however, is rarely associated with PCI. Few cases are reported in the world, and none has not been reported previously in Korea. A 67-year-old woman with polymyositis developed mild abdominal pain and abdominal distension during treatment with steroid and azathioprine. Radiographic findings including CT scan showed intraperitoneal free gas and intramural air, compatible with PCI. The patient's symptom and clinical findings improved after the treatment with antibiotics and high-dose oxygen therapy.
Aged ; Anti-Bacterial Agents/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Azathioprine/therapeutic use ; Cefotaxime/therapeutic use ; Female ; Humans ; Oxygen Inhalation Therapy ; Pneumatosis Cystoides Intestinalis/complications/*diagnosis/drug therapy ; Polymyositis/complications/*diagnosis/drug therapy ; Prednisolone/therapeutic use ; Radiography, Abdominal ; Tomography, X-Ray Computed

OBJECTIVES: Avascular necrosis of bone has been frequently documented in association with systemic lupus erythematosus and it has been suggested by many investigators that systemic factors may be implicated in its pathogenesis. In order to define the incidence, clinical feature and related risk factors of avascular necrosis in corticosteroid- treated rheumatic disease patients, we conducted this retrospective study. METHODS: Medical records of 278 patients with diagnoses of systemic lupus erythematosus (SLE), polymyositis/dermatomyositis, overlap syndrome comprising either of SLE, polymyositis, or dermatomyositis, and mixed connective tissue disease were reviewed with regards to the following: 1) duration of disease, risk factors of avascular necrosis, such as the presence of Raynaud phenomenon, small vessel vasculitis, alcoholism. 2) history of steroid treatment, including duration, initial dose, cumulative dose and mean daily dose during follow-up, cumulative dose and mean daily dose during the first year of disease, history of steroid pulse therapy, and history of cytotoxic drug therapy. 3) laboratory findings including false positive VDRL, lupus anticoagulant, anti-phospholipid antibody, and activated partial thromboplastin time. 4) Development of avascular necrosis, duration of disease, activity of disease at the time of diagnosis of avascular necrosis, and the site. RESULTS: Nineteen patients developed avascular necrosis leading to the incidence rate of 18.5/1,000 patient-year. Sites of involvement were hip in 16 cases(84.2%), talus in 2 cases(10.5% ), and phalanx, scaphoid, and humerus in 1 case(5.3% ), respectively. Fifty-eight percent of patients had involvement in more than one site. Presence of Raynaud phenomenon, small vessel vasculitis, history of cytotoxic therapy, history of steroid pulse therapy, cumulative dose and mean daily dose of steroid during follow-up and 1st year of diagnosis were not significantly different between the 2 groups. CONCLUSIONS: The incidence of avascular necrosis in our patient population was similar to that reported in SLE patients previously, but other risk factor including steroid dosage could not be identified.
Alcoholism ; Dermatomyositis ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Hip ; Humans ; Humerus ; Incidence ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Medical Records ; Mixed Connective Tissue Disease ; Necrosis* ; Osteonecrosis ; Partial Thromboplastin Time ; Polymyositis ; Raynaud Disease ; Research Personnel ; Retrospective Studies ; Rheumatic Diseases* ; Risk Factors ; Talus ; Vasculitis