We report a case of patient with documented SLE who displayed dysuria, gastrointestinal (GI) symptoms and renal insufficiency associated with the unusual occurrence of bilateral hydroureteronephrosis due to urterovesical junction stricture (obstructive uropathy). Pathologic investigations disclosed chronic interstitial cystitis (IC) with evidence of focal immune complex deposition in the blood vessel walls of the bladder. The GI symptoms and dysuria regressed with initial therapy for SLE with steroids. However, the persistent obstructive uropathy (OU) and renal insufficiency required bilateral nephrostomy followed by steroids plus intravenous pulse injection of cyclophosphamide. The obstructive uropathy was relieved even after removing the nephrostomy tube and renal function remained stable. Including this case, nineteen SLE patients associated with clinical and radiographic findings of OU were found in the world literature and reviewed to find any consistent pattern of clinical features. Most of the patients with OU in SLE were female (mean age, 31.7 yr) and orientals (63%), and had interstitial cystitis (89%) as a common underlying cause with concomitant involvement of the GI tract (89%) and WHO class IV or V advanced glomerulonephritis (67%). Despite the remarkable response (68%) to steroids in majority of OU patients associated with SLE, certain patients still required surgical correction (32%) and some even died (32%). OU, potentially reversible, was not an exception in patients with SLE, which might be overshadowed by other major organ involvement of SLE.
Adolescent ; Adult ; Female ; Human ; Lupus Erythematosus, Systemic/*complications/*pathology ; Urethral Obstruction/*complications/*pathology

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

Adolescent ; Child ; Female ; Humans ; Kidney ; pathology ; Lupus Erythematosus, Systemic ; complications ; pathology ; Male ; Sex Characteristics

Adolescent ; Heart Valve Diseases ; etiology ; Heart Valves ; pathology ; Humans ; Lupus Erythematosus, Systemic ; complications ; pathology ; Male

OBJECTIVES: To investigate the clinical characteristics, pathological features, treatment regimen, and prognosis of children with lupus nephritis (LN) and thrombotic microangiopathy (TMA), as well as the treatment outcome of these children and the clinical and pathological differences between LN children with TMA and those without TMA. METHODS: A retrospective analysis was conducted on 12 children with LN and TMA (TMA group) who were admitted to the Department of Nephrology, Children's Hospital of Nanjing Medical University, from December 2010 to December 2021. Twenty-four LN children without TMA who underwent renal biopsy during the same period were included as the non-TMA group. The two groups were compared in terms of clinical manifestations, laboratory examination results, and pathological results. RESULTS: Among the 12 children with TMA, 8 (67%) had hypertension and 3 (25%) progressed to stage 5 chronic kidney disease. Compared with the non-TMA group, the TMA group had more severe tubulointerstitial damage, a higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score at onset, and higher cholesterol levels (P<0.05). There were no significant differences between the two groups in the percentage of crescent bodies and the levels of hemoglobin and platelets (P>0.05). CONCLUSIONS There is a higher proportion of individuals with hypertension among the children with LN and TMA, as well as more severe tubulointerstitial damage. These children have a higher SLEDAI score and a higher cholesterol level.
Child ; Humans ; Lupus Nephritis/complications* ; Kidney/pathology* ; Retrospective Studies ; Thrombotic Microangiopathies/therapy* ; Prognosis ; Hypertension/complications* ; Cholesterol ; Lupus Erythematosus, Systemic

Adult ; Biopsy ; Fatty Liver ; etiology ; pathology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications

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

OBJECTIVETo summarize the clinical features and therapeutic approach of systemic lupus erythematosus (SLE) complicated by transverse myelitis (TM).

METHODSThe clinical characteristics, laboratory examinations, treatment and prognosis of 6 SLE cases with TM were retrospectively analyzed with review of the literatures.

RESULTSThe 6 patients consisted of 5 females and 1 male aged 14 to 36 years (mean 23 years). The mean duration from symptom onset of SLE to TM was 8 months (1 to 13 months). All the patients had lower limb hypodynamia, and 3 of them developed upper limb hypodynamia. MRI scanning of the spine identified lesions in the cervical spinal cord in 2 cases, thoracic lesions in 3 cases, and multiple involvement of the cervical, thoracic and lumbar cord in 1 case. Examination of the cerebrospinal fluid yielded no specific findings except for leukocytosis in 1 case and hypoglycemia in another. Five cases were treated with high-dose MP+CTX, and the other case was treated with MP (80 mg/day)+CTX. Five patients responded favorably to the treatment, while the other showed no obvious improvement.

CONCLUSIONTM is a rare complication of SLE affecting mostly young patients and occurring in the early stage of the disease. Early diagnosis and aggressive treatment might improve the prognosis.

Adolescent ; Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Magnetic Resonance Imaging ; Male ; Myelitis, Transverse ; complications ; Spinal Cord ; pathology ; Young Adult

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