Interstitial fibrosis increases the risk of end-stage kidney disease in patients with lupus nephritis.

OBJECTIVE: To evaluate the risk of end-stage kidney disease (ESKD) in lupus nephritis (LN) patients using tubulointerstitial lesion scores. METHODS: Clinical profiles and histopathological presentations of 151 biopsy-proven LN patients were retrospectively examined. Risk factors of ESKD based on characteristics and scoring of their tubulointerstitial lesions (e.g. interstitial inflammation [II], tubular atrophy [TA], and interstitial fibrosis [IF]) were analyzed. RESULTS: The mean age of 151 LN patients was 36 years old, and 136 (90.1%) were female. The LN cases examined included: class I/II (n = 3, 2%), class III/IV (n = 119, 78.8%), class V (n = 23, 15.2%), and class VI (n = 6, 4.0%). The mean serum creatinine level was 1.4 mg/dl. Tubulointerstitial lesions were recorded in 120 (79.5%) patients. Prior to receiving renal biopsy, 9 (6.0%) patients developed ESKD. During the follow-up period (mean, 58 months), an additional 47 patients (31.1%) progressed to ESKD. Multivariate analyses identified serum creatinine (hazard ratio [HR]: 1.7, 95% confidence interval [CI]: 1.42-2.03, p < 0.001) and IF (HR: 3.2, 95% CI: 1.58-6.49, p = 0.001) as independent risk factors of ESKD. Kaplan-Meier analysis further confirmed a heightened risk of ESKD associated with IF. CONCLUSION: Tubulointerstitial involvement is commonly observed in histopathological presentation of LN. However, IF, rather than II, or TA, was found to increase the risk of ESKD in our cohort. Therefore, to predict renal outcome in LN patients prior to adjusting immunosuppressive treatment, degree of IF should be reviewed.

Risk factors for mortality in systemic lupus erythematosus patients: Analysis of adult and pediatric cohorts in Taiwan.

BACKGROUND: Overall survival of systemic lupus erythematosus (SLE) patients significantly increased in recent decades, however, the relative risk of mortality is still high. Long-term survival outcome of pediatric SLE remains unclear. This study aims to explore the long-term survival rate and its predictors in patients with systemic lupus erythematosus (SLE). METHODS: A retrospective, hospital-based cohort study was performed between 2004 and 2018 in a tertiary referral medical center in Taiwan. Data on comorbidities, medications, and causes of admission were collected for risk factor analysis using time-dependent multivariate Cox proportional hazards models. RESULTS: A total of 2392 adults and 115 pediatric SLE patients were enrolled (female, n = 2157 and 95, respectively). The 10-year survival rates were 93.2%, 90.2%, 98.9%, and 100% in adult women, adult men, girls, and boys with SLE, respectively. The overall mortality rate was 2.09 case/100 patient-years (PY) for male SLE and 1.39 case/100 PY for female SLE patients. Male SLE patients did not have a statistically significantly higher mortality rate than female SLE patients in each age stratification. Infectious disease (n = 119), heart failure (n = 21), and cerebrovascular accident (n = 14) were the leading causes of death in adult SLE patients. Advanced age (hazard ratio [HR]: 1.04, 95% confidence interval [CI]: 1.03-1.05), treatment with mean dosage of systemic glucocorticoid equivalent to >10 mg/d of prednisolone (HR: 1.71, 95% CI: 1.14-2.57), comorbidities with malignancy (HR: 1.94, 95% CI: 1.22-3.09), chronic kidney disease (HR: 1.86, 95% CI: 1.25-2.77), hypertension (HR: 1.42, 95% CI: 1.01-1.98), and admission due to bacterial pneumonia (HR: 1.92, 95% CI: 1.12-3.31) and sepsis (HR: 2.78, 95% CI: 1.51-5.13) were independent risk factors for mortality in SLE patients. CONCLUSION: SLE patients with advanced age, malignancy, chronic kidney disease, hypertension, treated with a higher average dosage of glucocorticoids, and admission due to bacterial pneumonia and sepsis have an increased risk of mortality.

Systemic amyloidosis manifesting as a rare cause of hepatic failure.

In 1854, the term "amyloid" was first used in the description of a liver specimen at autopsy by Virchow. The kidneys and heart are the most commonly involved organs in amyloidosis; liver and gastrointestinal tract involvement is less common, and the symptoms are usually mild. Here, we report the case of a 57-year-old male patient who presented with oral hemorrhagic bullae, thrombocytopenia and jaundice. Disseminated intravascular coagulation profile was positive. Abdominal sonography showed ascites, and abdominal computed tomography disclosed heterogeneous enhancement of the liver, with focal low attenuation regions and splenomegaly with poor contrast enhancement. Liver decompensation was highly suspected. Diagnostic laparoscopy with liver biopsy and colonoscopic biopsy from the rectum were subsequently performed. Typical apple-green birefringence was demonstrated on polarized light microscopy by Congo red staining. Systemic amyloidosis was diagnosed and colchicine prescribed. However, liver function deteriorated and intermittent gastrointestinal bleeding was found during the patient's hospitalization. The patient died due to uncorrectable coagulopathy and massive gastrointestinal bleeding. The final diagnosis was idiopathic amyloidosis with hepatic failure. Although amyloidosis rarely presents with hepatic failure, it should be considered in patients with signs of liver decompensation. Clinicians should be aware of this rare but potentially lethal presentation and arrange appropriate treatment promptly.

Mycobacterium-associated lobular panniculitis, mimicking a rheumatoid nodule in a patient with rheumatoid arthritis.

Mycobacterium-associated lobular panniculitis can mimic a rheumatoid nodule and has been seldom reported in rheumatoid arthritis (RA). We describe a 69-year-old woman with RA who presented initially with fever and an indurated skin lesion on the right thigh. Lobular panniculitis was diagnosed after biopsy and was then treated with prednisolone. After this therapy, pulmonary infiltration developed and was later shown by transbronchial biopsy to be caused by Mycobacterium tuberculosis. The panniculitis skin lesion became smaller after prednisolone therapy and was further improved after antituberculosis drugs were added. Reexamination of the previously biopsied skin tissue disclosed acid-fast bacilli. Reactivation or new infection of M. tuberculosis is a current important issue in RA patients, especially after treatment with disease-modifying anti rheumatic drugs or antitumor necrosis factor agents. Mycobacterium-associated lobular panniculitis should be included in the differential diagnosis of indurated skin disorder in RA patients, and acid-fast staining or polymerase chain reaction examination of tuberculosis should be performed routinely on biopsied skin tissue.

Hemolytic uremic syndrome with ischemic glomerulonephropathy and obliterative vasculopathy in a systemic sclerosis patient treated with cyclosporine-A.

Hemolytic uremic syndrome (HUS) is not a commonly reported complication in post-transplantation patients treated with cyclosporine-A (CSA), and is extremely rare in systemic sclerosis (SSc) patients treated with this drug. CSA may contribute to the development of chronic ischemic glomerulonephropathy and vasculopathy, features not easily distinguished from SSc-related nephropathy. Here, we describe a 41-year-old Chinese man with diffuse-type SSc treated with CSA who developed thrombocytopenia, acute renal failure and hemolytic anemia and was diagnosed with HUS. Renal function and thrombocytopenia improved gradually after intensive treatment of plasma exchange (PE) and high-dose steroid therapy. After PE, renal biopsy showed ischemic glomerulonephropathy and obliterative vasculopathy. This case illustrates that PE can improve the hematological disorders and characteristic renal changes of HUS in SSc patients treated with CSA. However, this therapy may not be effective in normalizing serum creatinine level in SSc patients once CSA has triggered the normal kidney to develop glomerulonephropathy and vasculopathy with ischemic and sclerotic changes.

Recurrent autoimmune inner ear disease (AIED) and polyarteritis nodosa in a patient with large cell lung carcinoma.

Autoimmune inner ear disease (AIED) is a very rare disorder with distinct clinical features and can occur in patients with malignancy or autoimmune diseases. We report a 72-year-old male patient with polyarteritis nodosa treated continuously for 5 years with aggressive immunosuppressive drugs, including cyclophosphamide, who experienced three episodes of acute hearing loss during treatment. Organic lesions of the external and middle ear were excluded by repeated examinations, and if one subscribes to McCabe's (Ann Otol Rhinol Laryngol 88:585-589, 1979) definition of AIED, this condition must be considered as the likely cause of the hearing loss. During the period of treatment, three episodes of AIED occurred, and eventually, lung cancer developed. From the time relationship and clinical manifestations of neuropathy and livedo reticularis, the first episode of hearing loss was more likely to be related to vasculitis itself, while the third episode may well have been associated with the development of lung cancer given the dramatic improvement in the clinical condition following treatment of the tumor by excision and cancer chemotherapy. Coexistence of AIED, vasculitis, and malignancy in the same patient has only been reported infrequently, and our case suggests that this coexistence may not be coincidental. For those patients with autoimmune disease who are on long-term immunosuppressive drug therapy, active surveillance for a nascent malignant tumor should be exercised if AIED recurs or persists.

Conjunctival biopsy in sarcoidosis.

BACKGROUND: Conjunctival biopsy is considered to be a simple, safe and specific diagnostic procedure for sarcoidosis. This study was designed to determine the value of this procedure in Taiwan. METHODS: This study was conducted from December 2003 to April 2005 at the uveitis clinic of Taipei Veterans General Hospital. Blind sampling was conducted, obtaining a biopsy sample measuring 1 cm long by 3 mm wide from both lower fornices. A positive result was defined as the presence of non-caseating granuloma when other granuloma-forming processes had been excluded. RESULTS: Twenty-nine patients (7 men, 22 women) were enrolled. Mean age at diagnosis was 47.8 +/- 12.4 years. The most common initial symptom was eye-related problems in 19 (65.5%) patients. Of 58 biopsies, 15 (25.9%) specimens in 11 (37.9%) patients proved to be positive. Four patients experienced bilateral involvement; 7 patients had unilateral involvement. No prominent conjunctival nodules or follicles were noted. Gender, age, presence of uveitis, initial symptoms, and chest condition comparisons revealed no association between positive and negative conjunctival biopsies. CONCLUSION: Blind and bilateral conjunctival biopsy, due to its ease, safety and specificity, could be the first biopsy inpatients with clinical or chest X-ray abnormalities suggesting sarcoidosis. None of our patients with positive biopsy had nodular lesions.

Temporal arteritis.

Temporal arteritis, a chronic inflammatory vasculitis involving medium- and large-sized arteries, has rarely been reported in Asia. However, we report 2 cases, in which the patients initially presented with headache. Physical examination disclosed engorged, hard and palpable vessels in the temporal areas. Temporal-artery biopsy revealed 2 different types of arteritis: the multinucleated giant cell type and the panarteritis type without multinucleated giant cells. One patient was positive for immunoglobulin G anticardiolipin antibody. The pathologic findings of the different subsets of temporal arteritis, and the relationship between anticardiolipin antibody and the extent of vascular complications of temporal arteritis, are discussed.