Spontaneous pneumomediastnum has been reported only recently as a rare complication of dermatomyositis. We are reporting two cases of spontaneous pneumomediastinum in dermatomyositis. The diagnosis of dermatomyositis was based on the diagnostic criteria requiring symmetric weakness of limb-girdle muscles and anterior neck flexors, elevation of serum skeletal muscle enzymes, electromyograpic findings of inflammatory myopathy, positive findings on muscle biopsy and dermatologic features including heliotrope rash and Gottron s papule. Vasculitis may be the common denominator leading to the association of dermatomyositis and pneumomediasinum. In reported cases, the prognosis of pneumomediastinum seems unfavorable. Interestingly the 2 cases we are reporting resolved spontaneously without any recurrence.
Biopsy ; Dermatomyositis* ; Diagnosis ; Exanthema ; Lung Diseases, Interstitial ; Mediastinal Emphysema* ; Muscle, Skeletal ; Muscles ; Myositis ; Neck ; Prognosis ; Recurrence ; Vasculitis

Localized nodular myositis is an uncommon benign inflammatory myopathy of unkonwn cause affecting skeletal muscle and, presenting as a localized painful swelling within the soft tissue of an extremity. Histological examination reveals lymphocytic infiltration, scattered muscle fiber necrosis and regeneration, and interstitial fibrosis. MRI finding is an enhancement with increased signal intensity around the lesion. We report two cases of localized nodular myositis presenting as pseudothrobothrombophlebitis. We believe this is the first case report of localized nodular myositis in Korea.
Extremities ; Fibrosis ; Korea ; Magnetic Resonance Imaging ; Muscle, Skeletal ; Myositis* ; Necrosis ; Regeneration

Churg-Strauss syndrome(CSS) or allergic angiitis and granulomatosis is a disorder characterized by pulmonary and systemic small-vessel vasculitis, extravascular granulomas, and hypereosinophilia. It occurs in individuals with asthma and allergic rhinitis. The diagnosis of CSS is made on the basis of clinical and pathologic features. According to 1990 American College of Rheumatology(ACR) criteria for the classification of CSS, 6 criteria were developed. The presence of 4 or more of these criteria yielded a sensitivity of 85% and a specificity of 99. 7%. We describe a case of CSS in a 62-year-old female who met all of 1990 ACR criteria, but presented as like a as rheumatoid arthritis initially. Clinical symptoms, laboratory and roentgenographic findings gradually responded to high dose prednisolone treatment and resolved 3 weeks later. After discharge, she has been treated with oral prednisolone in a tapering course. Although polyarthritis with eosinophilia, vasculitis, and neuropathy are clinical manifestations of rheumatoid arthritis, those maniestations frequently occur during the vasculitic phase of the CSS. This case suggests that thorough differentiation of extra-articular manifestations of RA from clinical manifestations of CSS is considered when we meet a patient who have polyarthritis and vasculitis.
Arthritis ; Arthritis, Rheumatoid* ; Asthma ; Churg-Strauss Syndrome ; Classification ; Diagnosis ; Eosinophilia ; Female ; Granuloma ; Humans ; Middle Aged ; Prednisolone ; Rhinitis ; Sensitivity and Specificity ; Vasculitis

Rice bodies are numerous small fibrinous, cartilaginous-like materials which were first described in tuberculous joints. Rice bodies are common findings in joints afflicted with rheumatoid arthritis or other seronegative arthropathy. But less commonly, those can be seen in periarticular bursae or at the sites of tendon or ligament insertion. Rice bodies, thought to be a nonspecific response to synovial inflammation, probably evolve from ischemia in a proliferative synovium. Some of these bodies contain a core of collagen with a mantle of fibrin, others contain only fibrin. Subacromial bursa are sometimes involved in the patients with rheumatoid arthritis and can reach impressive dimensions before becoming clinically detectable because significant constrain is lacking. Subacromial arthrography or magnetic resonance imaging accurately delineated the existence of cartilaginous loose bodies before surgical exision. We experienced a man with rheumatoid arthritis who had massive subacromial bursitis with rice bodies. His shoulder had been swollen since 5 years ago. He felt no pain and had only mild limitation of motion. Subacromial arthrography or magnetic resonance imaging of right shoulder showed multiple rice bodies in enlarged subacromial bursa. Operation finding showed a large encapsulated mass in the subacromial bursa, and hundreds of fibrinous rice bodies were revealed, which were resected. We report this patient with a review of literatures.
Arthritis, Rheumatoid* ; Arthrography ; Bursitis* ; Collagen ; Fibrin ; Humans ; Inflammation ; Ischemia ; Joints ; Ligaments ; Magnetic Resonance Imaging ; Shoulder ; Synovial Membrane ; Tendons

The use of oral methotrexate in a low dose given once weekly has become the mainstay of therapy for active and sustained rheumatoid arthritis. Pneumonitis can be expected to occur in patients taking low doses of methotrexate for rheumatoid arthritis. The pathology suggests that methotrexate pneumonitis is a hypersensitivity reaction although arguments have been put forth that it is idiosyncratic. Treatment of presumed methotrexate pneumonitis, even while waiting for special stains, cultures, or tissue sections from bronchoscopic biopsy, should be glucocorticoids given intravenously or by mouth. Empirical antibiotic treatment can be used until infectious causes are ruled out. In recent years there has been an increase in the number of reports of pulmonary complications associated with low-dose methotrexate therapy for rheumatic diseases. Among these complications interstitial pneumonitis has been most often reported (more than 35 cases since the first report in 1983). We report a case of methotrexateassociated pulmonary complication in rheumatoid arthritis confirmed by transbronchoscopic lung biopsy, which resolved by treatment of corticosteroid therapy.
Arthritis, Rheumatoid* ; Biopsy ; Coloring Agents ; Glucocorticoids ; Humans ; Hypersensitivity ; Lung ; Lung Diseases, Interstitial* ; Methotrexate* ; Mouth ; Pathology ; Pneumonia ; Rheumatic Diseases

We experienced a case of diffuse idiopathic skeletal hyperostosis(DISH) associated with ossification of the posterior longitudinal ligament. A 58-year old man presented with clumsiness and hypesthesia of both hands. He showed weakness of sensory defit and weakness of motor function of hands. The lateral radiograph of cervical spine showed anterior hyperostosis and ossification of the posterior longitudinal ligament. A magnetic resonance image of cervical spine revealed a low-density defect posterior to cervical spine with evidence of spinal stenosis. Ossification of the posterior longitudinal ligament is a rare manifestation associated with DISH, and we report this case with a review of the literature.
Hand ; Humans ; Hyperostosis ; Hyperostosis, Diffuse Idiopathic Skeletal* ; Hypesthesia ; Ligaments* ; Longitudinal Ligaments ; Middle Aged ; Ossification of Posterior Longitudinal Ligament ; Spinal Stenosis ; Spine

Sacroiliitis is a prototyical feature of the seronegative spondyloarthropathies. It has been reported rarely in patients with systemic lupus erythematosus. We report a case of bilateral sacroiliitis in a 26 year-old female who presented 5 out of 11 ARA criteria for systemic lupus erythematosus. She complained of right low back pain since 1 week after delivery. Plain roentgenogram, computed tomogram and magnetic resonance imaging revealed no evidence of subluxation or pyogenic infection that may be possibly related to delivery, but bilateral sacroiliitis especially more severe in the right side that showed concordantly increased uptake on bone scan. Also she had neither HLA DR-3 nor B-27 antigens and had no other clinical findings of limitation of motion of spine and chest expansion, which suggest that there is a less likelihood of coexistent ankylosing spondylitis. In this case, we suggested that sacroiliitis may be a infrequent manifestation of systemic lupus erythematosus.
Adult ; Female ; Humans ; Low Back Pain ; Lupus Erythematosus, Systemic* ; Magnetic Resonance Imaging ; Sacroiliitis* ; Spine ; Spondylarthropathies ; Spondylitis, Ankylosing ; Thorax

A 42-year-old man presented with severe pancytopenia and uncontrolled epistaxis. The diagnosis of SLE was made and the pancytopenia was found to be due to myelofibrosis. The pulse therapy with methylprednisolone and maintenance therapy with prednisolone reversed both pancytopenia and myelofibrosis. Although myelofibrosis has been described in SLE, this coexistence must be very rare since there has been only 19 cases showing this combination. We report a case of SLE with myelofibrosis which was reversed by the treatment with glucocorticoid.
Adult ; Diagnosis ; Epistaxis ; Humans ; Lupus Erythematosus, Systemic* ; Methylprednisolone ; Pancytopenia ; Prednisolone ; Primary Myelofibrosis*

The antiphospholipid syndrome is characterized by arterial thrombosis, venous thrombosis, pregnancy wastage, and thrombocytopenia associated with a persis tently positive lupus anticoagulant and/or moderate to high positive anticardiolipin antibodies(IgG or IgM). The antiphospholipid antibodies have been detected in many medical conditions, but the antiphospholipid syndrome (APS) has mainly been restricted to the primary antiphospholipid syndrome and APS associated with systemic lupus erythematosus. Rarely, the APS has been reported in other autoimmune disorders in the literature. We describe a woman with a limited form of scleroderma and the APS manifested by complete occlusion of left axillary artery with probable thrombotic occlusive nature, thrombocytope nia, prolonged aPTT, and persistently positive lupus anticoagulant.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Axillary Artery ; Female ; Humans ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Pregnancy ; Scleroderma, Limited* ; Thrombocytopenia ; Thrombosis ; Venous Thrombosis

Systemic lupus erythematosus (SLE) is a multisystemic disease that can involve the gastrointestinal tract, liver, and biliary system. Symptomatic pancreatic involvement, however, has rarely been reported. It may be part of the primary disease process, such as vasculitic or autoimmune etiology, or associated with drug therapy, in particular corticosteroid. We report here a lupus patient who developed severe pancreatitis within 30 hours of initiation of corticosteroid therapy; we also discuss the relation between pancreatitis and systemic lupus erythematosus.
Biliary Tract ; Drug Therapy ; Gastrointestinal Tract ; Humans ; Liver ; Lupus Erythematosus, Systemic* ; Pancreatitis*