Polymyositis (PM) is a chronic inflammatory disease that predominantly affects muscles. Systemic organ involvement, including the respiratory and gastrointestinal tracts, is frequently observed in PM, but renal involvement is rare. Herein, we report the case of a 56-year-old woman presenting with weight gain, edema, and generalized myalgia. Laboratory tests revealed elevated creatinine kinase level, hypoalbuminemia, and proteinuria. Histopathological examination of muscle biopsy revealed inflammatory myositis, and a renal biopsy confirmed immunoglobulin A (IgA) nephropathy. Based on the clinico-pathological results, the patient was diagnosed with PM with IgA nephropathy. This is a report of a rare occurrence of IgA nephropathy in a patient with PM presenting with chronic glomerulonephritis.
Biopsy ; Creatinine ; Edema ; Female ; Gastrointestinal Tract ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Hypoalbuminemia ; Immunoglobulin A* ; Immunoglobulins* ; Middle Aged ; Muscles ; Myalgia ; Myositis ; Phosphotransferases ; Polymyositis* ; Proteinuria ; Weight Gain

PURPOSE: Periocular tuberculosis is common in the form of conjunctival tuberculosis or uveitis; however, orbital tuberculosis is very rare. We report a case of tuberculosis diagnosed based on bilateral orbital masses. CASE SUMMARY: A 64-year-old male with a history of diabetes, hypertension, and chronic obstructive pulmonary disease presented with diplopia. His best-corrected visual acuity was 1.0, and intraocular pressure was 16 mmHg in both eyes. Bilateral proptosis was noted, and inferior orbital masses were palpated in both eyes. On orbital computed tomography, irregular masses were surrounding both globes in the extraconal space. Incisional biopsy of the bilateral orbital masses was performed under local anesthesia. Pathologic examination revealed chronic granulomatous inflammation accompanied by caseous necrosis. The patient was diagnosed with bilateral orbital tuberculosis and underwent triple therapy with isoniazid, rifampin, and ethambutol for 12 months. The orbital masses completely resolved, and there was no recurrence or complication through 12 months after treatment. CONCLUSIONS: To our knowledge, bilateral orbital tuberculosis has not been reported yet. Herein, we report a case of orbital tuberculosis presenting as bilateral orbital masses.
Anesthesia, Local ; Biopsy ; Diplopia ; Ethambutol ; Exophthalmos ; Humans ; Hypertension ; Inflammation ; Intraocular Pressure ; Isoniazid ; Male ; Middle Aged ; Necrosis ; Orbit* ; Pulmonary Disease, Chronic Obstructive ; Recurrence ; Rifampin ; Tuberculosis* ; Uveitis ; Visual Acuity

We need to establish an informative guideline to increase inter-institutional and inter-observer reproducibility of renal transplant diagnosis, and to improve the diagnostic ability of pathologists in Korea. A first nation-wide survey for renal transplant pathology was conducted by Renal Pathology Study Group of the Korean Society of Pathologists in 2016, to provide the continued excellence in the transplantation pathology laboratory, and to improve the diagnostic ability for the best treatment of transplant patients. This survey revealed the significant variations in scale, work load and biopsy indications for the renal transplant pathology in various institutions in Korea. The Banff classification were used by all institutions for the diagnosis of renal transplant pathology, but different formats were used: most institutions (70%) used the “2013 Banff classification” while the others were using “2007 Banff classification” (20%) or even older formats. In daily diagnostic practice of the renal allografts, difficulties that pathologists encounter were quite diverse due to different environments they work in. Most respondents agreed that standardized diagnostic practice guidelines, regular education on renal transplant pathology and convenient ways of consultation are further needed. We are currently working toward the enhancement of the expertise of renal pathologists and to increase inter-institutional and inter-observer reproducibility by 1) development of a set of virtual slides of renal allograft biopsies for the training, 2) validation and gathering expert's consensus on the core variables of rejection diagnosis by using virtual slides, and 3) continued education by the developed virtual slide atlas.
Allografts ; Biopsy ; Classification ; Consensus ; Diagnosis ; Education ; Humans ; Kidney ; Korea* ; Pathology* ; Surveys and Questionnaires ; Transplantation

Bladder urothelial carcinoma typically occurs in individuals in their sixties or seventies: it rarely occurs in persons <20 years old. Moreover, in young patients, bladder urothelial carcinoma lesions are reported to be solitary and nonmuscle invasive, with low malignant potential. However, 13% of lesions in this age group are reported to be noninvasive high-grade papillary urothelial carcinoma but these are extremely rare in children <15 years. There is no specific consensus regarding treatment of bladder urothelial carcinoma in children and adolescent. In particular, method has been suggested for follow-up of high-grade bladder urothelial carcinoma. Therefore we report our experience of one case the, discuss surveillance methods, and provide a brief review of the literature.
Adolescent ; Child* ; Consensus ; Follow-Up Studies ; Humans ; Methods ; Pediatrics ; Urinary Bladder*

BACKGROUND/AIMS: Immunoglobulin A nephropathy (IgAN) is a generally progressive disease, even in patients with favorable prognostic features. In this study, we aimed to investigate the antiproteinuric effect and tolerability of low-dose valsartan (an angiotensin II receptor blocker) therapy in normotensive IgAN patients with minimal proteinuria of less than 0.5 to 1.0 g/day. METHODS: Normotensive IgAN patients, who had persistent proteinuria with a spot urine protein-to-creatinine ratio of 0.3 to 1.0 mg/mg creatinine, were recruited from five hospitals and randomly assigned to either 40 mg of valsartan as the low-dose group or 80 mg of valsartan as the regular-dose group. Clinical and laboratory data were collected at baseline, and at 4, 8, 12, and 24 weeks after valsartan therapy. RESULTS: Forty-three patients (low-dose group, n = 23; regular-dose group, n = 20) were enrolled in the study. Proteinuria decreased significantly not only in the regular-dose group but also in the low-dose group. The change in urine protein-to-creatinine ratio at week 24 was -41.3% +/- 26.1% (p < 0.001) in the regular-dose group and -21.1% +/- 45.1% (p = 0.005) in the low-dose group. In the low-dose group, blood pressure was constant throughout the study period, and there was no symptomatic hypotension. In the regular-dose group, blood pressure decreased at weeks 8 and 12. No significant change in glomerular filtration rate, serum creatinine level, or serum potassium level was observed during the study period. CONCLUSIONS: Our results suggest that low-dose valsartan can significantly reduce proteinuria without causing any intolerability in normotensive IgAN patients with minimal proteinuria.
Adult ; Angiotensin II Type 1 Receptor Blockers/*administration & dosage/adverse effects ; Biomarkers/urine ; Blood Pressure ; Creatinine/urine ; Female ; Glomerulonephritis, IGA/diagnosis/*drug therapy/physiopathology/urine ; Humans ; Male ; Middle Aged ; Prospective Studies ; Proteinuria/diagnosis/*drug therapy/physiopathology/urine ; Republic of Korea ; Time Factors ; Treatment Outcome ; Valsartan/*administration & dosage/adverse effects

Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin, especially in the central nervous system (CNS). Reported herein is a case of SFT of CNS in a 63-year-old female patient who had confused mentality, without other neurological deficit. The brain MRI showed an ovoid mass in the right frontal lobe. The tumor was surgically removed grossly and totally, and the pathologic diagnosis was SFT. At 55 months after the surgery, the tumor recurred at the primary site and at an adjacent area. A second operation was thus done, and the tumor was again surgically removed grossly and totally. The pathologic diagnosis was the same as the previous, but the Ki-67 index was elevated. Ten months later, two small recurring tumors in the right frontal skull base were found in the follow-up MRI. It was decided that radiation therapy be done, and MRI was done again 3 months later. In the follow-up MRI, the size of the recurring mass was found to have decreased, and the patient did not manifest any significant symptom. Follow-up will again be done 18 months after the second surgery.
Brain ; Central Nervous System* ; Diagnosis ; Female ; Follow-Up Studies ; Frontal Lobe ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Skull Base ; Solitary Fibrous Tumors*

Behcet's disease is a systemic inflammatory disorder of unknown etiology, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Renal involvement is rare in patients with Behcet's disease particularly immunoglobulin A (IgA) nephropathy. Other autoimmune diseases have been associated with increased risk of malignancy, but not Behcet's disease. Some cases of Behcet's disease accompanied by bladder cancer, thyroid cancer, stomach cancer, or hematologic malignancies have been reported. However, to the best of our knowledge, co-occurrence of Behcet's diseases with thymic carcinoma has not yet been reported. We experienced a 49-year-old male patient who had been treated for Behcet disease and IgA nephropathy, who presented with a large mediastinal mass on chest x-ray. After thymectomy, he was diagnosed with thymic carcinoma with complete resection.
Autoimmune Diseases ; Behcet Syndrome ; Glomerulonephritis, IGA* ; Hematologic Neoplasms ; Humans ; Immunoglobulin A ; Male ; Middle Aged ; Skin ; Stomach Neoplasms ; Stomatitis, Aphthous ; Thorax ; Thymectomy ; Thymoma* ; Thyroid Neoplasms ; Ulcer ; Urinary Bladder Neoplasms ; Uveitis

The olfactory groove schwannoma is a quite rare tumor. We report a case of a 49-year-old woman with an olfactory groove schwannoma attached to the cribriform plate without olfactory dysfunction. She had no specific neurological symptoms other than a headache, and resection of the tumor showed it to be a schwannoma. About 19 months after the operation, a follow-up MRI showed no evidence of tumor recurrence. Surgical resection through subfrontal approach could be one of the curative modality in managing an olfactory groove schwannoma. An olfactory groove schwannoma should be considered in the differential diagnosis of anterior skull base tumors.
Diagnosis, Differential ; Ethmoid Bone* ; Female ; Follow-Up Studies ; Headache ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neurilemmoma* ; Prefrontal Cortex ; Recurrence ; Skull Base

Ancient schwannoma is a variant of schwannoma and is characterized slowly growing tumor with degenerative change. And it is reported that schwannoma is relatively rare in extensor area. As a rare cause of solitary ancient schwannoma in extensor area of upper arm, we report it.
Arm ; Neurilemmoma

Glomerulonephritis occurs as a rare form of renal manifestation in Plasmodium falciparum malaria. Herein, we report a case of falciparum malaria-associated IgA nephropathy for the first time. A 49-yr old male who had been to East Africa was diagnosed with Plasmodium falciparum malaria. Microhematuria and proteinuria along with acute kidney injury developed during the course of the disease. Kidney biopsy showed mesangial proliferation and IgA deposits with tubulointerstitial inflammation. Laboratory tests after recovery from malaria showed disappearance of urinary abnormalities and normalization of kidney function. Our findings suggest that malaria infection might be associated with IgA nephropathy.
Acute Kidney Injury/etiology/pathology ; Antimalarials/therapeutic use ; Creatinine/blood ; Glomerulonephritis, IGA/*diagnosis/*etiology ; Hematuria/etiology ; Humans ; Immunoglobulin A/*metabolism ; Malaria/*complications/drug therapy/*pathology ; Male ; Middle Aged ; Plasmodium falciparum/*isolation & purification ; Proteinuria/etiology ; Quinine/therapeutic use