Localized Gastrocnemius Myositis in Crohn's Disease

We describe a case of localized gastrocnemius myositis which developed with flare-up of Crohn's disease. A 21-year old male patient with an 8-year history of Crohn's disease presented with pain and tenderness in both calves without recent abdominal symptoms. Electromyography and gastrocnemius muscle biopsy revealed evidence of inflammatory myositis. Magnetic resonance imaging (MRI) showed bilateral symmetrical diffuse increased signal intensity in T2 weighted images in both gastrocnemius muscles and patchy contrast enhancement. Subsequent gastrointestinal investigation revealed active inflammation of colon with multiple pseudopolyps and enteroenteric fistula on which we commenced oral prednisolone of 30 mg daily. His pain on both calves was improved and muscle enzymes became normal. Following dose reduction of prednisolone, azathioprine 50 mg daily was started considering the patient's active Crohn's disease on endoscopic findings prior to the development of overt abdominal symptoms. This is the first case report of localized gastrocnemius myositis associated with Crohn's disease described in Korea. Calf myositis responded to corticosteroid well and did not recur with maintenance therapy using azathioprine and mesalazine.

Aspergillus-Associated Cerebral Aneurysm Successfully Treated by Endovascular and Surgical Intervention with Voriconazole in Lupus Nephritis Patient

During the last five decades, long-term therapy with immunosuppressive agents such as pulse cyclophosphamide in conjunction with high-dose corticosteroids has enhanced both patient survival and renal survival in patients with diffuse proliferative lupus nephritis. Nevertheless, severe side effects such as infectious complications remain the main cause of morbidity and mortality. Central nervous system aspergillosis is uncommon but life-threatening in lupus patients. In this single-patient case study, carotid aneurysm with sphenoidal sinusitis was suspected when severe epistaxis occurred during cyclophosphamide pulse therapy. With anti-fungal therapy, a graft stent was successfully deployed to the aneurysm and specimens of sphenoidal mucosa showed typical hyphae, indicating aspergillosis. Three months after stopping voriconazole treatment, two cerebral aneurysms that were revealed on MR images were successfully removed by aneurysmal clipping. The patient remained alive at one-year follow-up with lupus nephritis in remission. The rarity and high mortality of aspergillus-related fungal aneurysms have led to most cases being recognized postmortem. However, such aneurysms must be diagnosed early to prevent fatal complications by performing appropriate management such as surgical procedure or endovascular intervention.

A Case of Caplan's Syndrome Presenting as Acute Onset Polyarthritis

Caplan originally described distinctive pulmonary nodules in miners who had suffered from rheumatoid arthritis. Later, the pulmonary nodules, together with a history of rheumatoid arthritis and exposure to inorganic dust were called Caplan's syndrome. This syndrome has been described by case reports in many countries but only two cases have been reported in Korea up to now. The patient in this case report was a 70-years-old man who had worked in the construction field for 20 years mainly demolishing buildings. He was diagnosed with silicosis by a lung biopsy 1 year prior to admission. He suddenly developed arthralgia and morning stiffness in multiple joints 2 weeks prior to admission. Chest imaging revealed aggravation of the bilateral pulmonary nodules. He was diagnosed with seropositive rheumatoid arthritis. The lung nodules, arthralgia, and morning stiffness improved clinically after treatment with the corticosteroid and disease modifying anti-rheumatic drugs.

A Case of Churg-Strauss Syndrome with Endomyocardial Fibrosis / 대한류마티스학회지

Cardiac involvement is an important cause of mortality in patients with Churg-Strauss syndrome. The typical cardiac presentation of Churg-Strauss syndrome includes pericarditis, myocarditis, and cardiomyopathy. Endomyocardial fibrosis has rarely been described in patients with Churg-Strauss syndrome. We experienced a patient with Churg-Strauss syndrome who exhibited exertional dyspnea and endomyocardial fibrosis visualized as delayed enhancement on cardiac magnetic resonance imaging (MRI). After glucocorticoid treatment, the patient's symptom resolved, and the eosinophil count decreased to normal. Nine months later, the delayed-enhanced lesion on the cardiac MRI nearly disappeared. Here, we report a case of endomyocardial fibrosis in a patient with Churg-Strauss syndrome with a literature review.

A Case of Typical Cogan's Syndrome with Aortitis / 대한류마티스학회지

Cogan's syndrome can accompany a variety of systemic vasculitides including aortitis. A 45-year-old woman with a history of typical Cogan's syndrome presented with orthopnea and exertional dyspnea. Echocardiography demonstrated severe aortic valve insufficiency. Computed tomographic angiography demonstrated active vasculitis affecting the ascending and descending aortas and also showed stenosis of the left subclavian artery, both renal arteries, the celiac axis, the superior mesenteric artery, and the right common iliac artery. She received high dose corticosteroid and then underwent an aortic valve replacement. This is the first case of Cogan's syndrome with aortitis in Korea.