OBJECTIVE: To improve the understanding of lupus cystitis. METHODS: Clinical manifestations, laboratory Results , and image information of 2 cases of lupus cystitis were analysed retrospectively, and another 6 cases in the literature were reviewed. RESULTS: Two patients were female. The urinary symptoms followed the gastrointestinal symptoms. Ureterectasia and hydronephrosis were detected in both patients, and intestinal pseudo-obstruction was detected in one patient. In the 6 cases from the literature, ureterectasia and hydronephrosis were detected in all patients, and intestinal pseudo-obstruction was detected in 4. CONCLUSION The possibility of lupus cystitis should be considered when lupus patients complain of urinary or bowel symptoms. Glucocorticoid and immunodepressant are effective for lupus cystitis.
Adolescent ; Adult ; Cystitis ; complications ; diagnosis ; Female ; Humans ; Hydronephrosis ; etiology ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; Ureter ; pathology

A 16-year-old female patient with systemic lupus erythematosus (SLE) presented with abdominal pain and oliguria, after one month of hospital treatment with methylprednisolone and cyclophosphamide. Computed tomography of the abdomen revealed the calcification of bilateral superior segmental ureteral wall. Computed tomography urography revealed stenosis of bilateral ureters. This calcification may be associated with lupus-induced inflammatory reaction of ureteral endomembrane. By receiving the ureteral catheters, her abdominal pain and oliguria had been completely resolved. After discharge, she underwent the surgery of cutaneous ureterostomy in the local hospital. During two years follow-up, her condition is stable without any symptom of the urinary system.
Adolescent ; Calcinosis ; diagnosis ; etiology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Ureter ; pathology

We report on a 13-year-old female with systemic lupus erythematosus (SLE) who exhibited symptoms of severe migraine and familial moyamoya disease. Cerebral magnetic resonance angiography (MRA) showed stenosis and occlusion of the bilateral internal carotid arteries associated with the development of collateral circulation (moyamoya vessels). In a child, as in this case, headaches with cerebral infarction associated with moyamoya disease are unusual. Few cases of SLE associated with familial moyamoya disease have been reported, with no previous reports of such cases from Korea. There were no evidences of antiphospholipid syndrome, and activity of SLE or other risk factors for cerebral occlusion were also absent.
Adolescent ; Carotid Artery, Internal/pathology ; Female ; Human ; Lupus Erythematosus, Systemic/*complications ; Magnetic Resonance Angiography ; Migraine/etiology ; Moyamoya Disease/*diagnosis/*genetics

Antibodies, Antineutrophil Cytoplasmic ; blood ; Biopsy ; Child ; Diagnosis, Differential ; Female ; Humans ; Kidney Glomerulus ; pathology ; Lupus Erythematosus, Systemic ; complications ; immunology ; Lupus Nephritis ; diagnosis ; etiology ; immunology ; pathology ; Necrosis

BACKGROUND: Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBehcet's disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients. METHODS: Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study. We retrospectively analyzed the clinical and brain MRI data. The neuropsychiatric manifestations were classified in both groups according to the new American College of Rheumatology nomenclature for NPSLE. RESULTS: The diffuse symptoms that included mood disorders, psychosis, confusion, cognitive dysfunctions, generalized seizures and headaches other than migraine or cluster headaches were more commonly observed in the NPSLE patients, while the frequency of focal diseases such as cranial neuropathy tended to be higher in the NBD patients. The brain MRI revealed that the NBD patients had more abnormalities in the brain stem than did the NPSLE patients. Most of the patients improved, at least partially, after being treated with glucocorticoid and/or immune suppressants. However, the disease course differed significantly between the two groups. There were more episodic cases in the NPSLE group of patients, while there were more remittent cases in the NBD group of patients. CONCLUSION: NPSLE had a tendency to cause diffuse neuropsychiatric manifestations, and it has a different predilection of brain lesions compared with NBD. The NBD patients showed a poorer outcome than did the NPSLE patients, suggesting that different therapeutic strategies for the two diseases need to be considered.
Adult ; Behcet Syndrome/complications/*diagnosis/pathology ; Brain/*pathology ; Female ; Humans ; Lupus Erythematosus, Systemic/complications/diagnosis/pathology ; Lupus Vasculitis, Central Nervous System/complications/*diagnosis/pathology ; *Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies ; Risk Factors

Overlap of autoimmune hepatitis and systemic lupus erythematosus (SLE) is a comparatively rare condition. Although both autoimmune hepatitis and SLE can share common autoimmune features such as polyarthralgia, hypergammaglobulinemia and positive ANA, it has been considered as two different entities. We report a case of anti-LKM1 positive autoimmune hepatitis who developed SLE two years later. The presence of interface hepatitis with lymphoplasma cell infiltrates and rosette formation points to the autoimmune hepatitis rather than SLE hepatitis. Autoimmune hepatitis is infrequently accompanied by SLE, therefore, it could be recommended to investigate for SLE in patients with autoimmune hepatitis.
Antibodies, Antinuclear/analysis ; Autoantibodies/*analysis ; Echocardiography ; Female ; Hepatitis, Autoimmune/complications/*diagnosis/immunology ; Humans ; Liver/pathology ; Lupus Erythematosus, Systemic/complications/*diagnosis/immunology ; Young Adult

Thyroglossal duct carcinoma is a malignant tumor which occurs in the thyroglossal duct cyst. The incidence of thyroglossal duct carcinoma has been reported as approximately 1%. Up to now, just about 250 cases of thyroglossal duct carcinoma have been reported in the literature,most of which are single case reports and small case series. In most cases, the diagnosis of the thyroglossal duct carcinoma is not made until the histologic examination after surgery operation. The preoperative examination such as CT or fine needle aspiration cytology can help the preoperative diagnosis. But the surgical treatment for the thyroglossal duct carcinoma is still controversial. Now we report a case of a thyroglossal duct carcinoma combined with systemic lupus erythematosus. The patient herself found an anterior neck mass in the median submental region one year ago. The preoperative CT examination suggested thyroglossal duct cyst with pouch canceration(papillary carcinoma). Then she underwent a Sistrunk procedure and level I neck dissection, and the histopathological diagnosis was thyroglossal duct carcinoma. The patient was treated with levothyroxine therapy at suppressive dose after the surgery. Now the patient is at regular follow-up with no relapse occur.
Biopsy, Fine-Needle ; Carcinoma, Papillary ; complications ; diagnosis ; pathology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; pathology ; Neck Dissection ; Skin ; Thyroglossal Cyst ; complications ; diagnosis ; pathology ; Thyroid Neoplasms ; complications ; diagnosis ; pathology

Visceral leishmaniasis (VL) is a life-threatening infection caused by Leishmania species. In addition to typical clinical findings as fever, hepatosplenomegaly, and cachexia, VL is associated with autoimmune phenomena. To date, VL mimicking or exacerbating various autoimmune diseases have been described, including systemic lupus erythematosus (SLE), rheumatoid arthritis, and autoimmune hepatitis (AIH). Herein, we presented a patient with VL who had overlapping clinical features with SLE, AIH, as well as antimitochondrial antibody (AMA-M2) positive primary biliary cirrhosis.
Adult ; Autoantibodies/blood ; Bone Marrow/pathology ; Cytological Techniques ; Diagnosis, Differential ; Female ; Hepatitis, Autoimmune/complications/*pathology ; Histocytochemistry ; Humans ; Leishmaniasis, Visceral/complications/*diagnosis/*pathology ; Liver/pathology ; Liver Cirrhosis, Biliary/complications/*pathology ; Lupus Erythematosus, Systemic/complications/*pathology

Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.
Anti-Infective Agents/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Lupus Erythematosus, Systemic/*diagnosis/etiology/pathology ; Middle Aged ; Recurrence ; Skin/pathology ; Urticaria/complications/*diagnosis ; Vasculitis, Hypersensitivity/complications/*diagnosis/pathology

Lupus enteritis is a rare, severe complication of systemic lupus erythematosus (SLE), needing prompt diagnosis and proper management. However, SLE rarely presents as lupus enteritis at the time of initial diagnosis. Thus, delayed diagnosis and misdiagnosis are common. We report a case of a 25-year-old woman with lupus panenteritis. The patient had multiple hospitalizations for abdominal pain, nausea, and diarrhea, initially without any other symptoms suggestive of SLE, but was later observed to have malar rash and oral ulcers. Laboratory investigations were compatible with SLE, including positive antinuclear antibody (1:320) with speckled pattern. CT revealed diffuse hypodense submucosal thickening of the stomach, the entire small bowel, colon, appendix, and rectum. Treatment with high-dose corticosteroids followed by maintenance therapy with mycophenolate mofetil, hydroxychloroquine, and azathioprine resulted in clinical improvement. Diagnosis of lupus enteritis requires a high index of suspicion given the low incidence and nonspecific clinical findings.
Abdominal Pain/complications ; Adrenal Cortex Hormones/therapeutic use ; Adult ; Brain/diagnostic imaging ; Diagnosis, Differential ; Diarrhea/complications ; Endoscopy, Gastrointestinal ; Enteritis/pathology ; Female ; Humans ; Lupus Erythematosus, Systemic/complications/*diagnosis/drug therapy ; Magnetic Resonance Imaging ; Nausea/complications ; Tomography, X-Ray Computed