scholarly journals ANCA-associated vasculitis overlaps with systemic sclerosis: a case report and literature review

2021 ◽  
Vol 26 (1) ◽  
Author(s):  
Rui Wu ◽  
Jiang Su ◽  
Yu-rong Zou ◽  
Jing Zhu

Abstract Background Systemic sclerosis (SSc) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) both affect the kidney and may cause renal failure. Treatment of AAV is dramatically different from that of SSc renal crisis (SRC). Kidney biopsy is not recommended for diagnosing SRC, but it is the only reliable diagnostic method for AAV. Case presentation Here, a 49-year-old male patient with diffuse SSc presented with acute renal insufficiency and detectable ANCA with myeloperoxidase-specific antibodies. A renal biopsy revealed necrotizing glomerulonephritis and was consistent with AAV. This finding confirms the existence of AAV and SSc overlap syndrome. The patient was treated with intravenous methylprednisolone, intravenous cyclophosphamide, tandem membrane plasma exchange, and hemodialysis. After treatment, his clinical symptoms remained stable, and his creatinine and C-reactive protein (CRP) levels have remained normalized as of his most recent follow-up after hospital discharge. Conclusions AAV can overlap with SSc; although this condition is rare, it is associated with considerable morbidity and mortality. Therefore, it is critical to recognize AAV in the setting of worsening renal function due to SSs and provide appropriate treatment. Several clinical features are suggestive of AAV rather than SRC, but renal biopsy is required for accurate diagnosis.

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Athina Nikolarakou ◽  
Dana Dumitriu ◽  
Pierre-Louis Docquier

Primary arthritis of chondrosternal joint is very rare and occurs in infants less than 18 months of age. Presentation is most often subacute but may be acute. Child presents with a parasternal mass with history of fever and/or local signs of infection. Clinical symptoms vary from a painless noninflammatory to a painful mass with local tenderness and swelling, while fever may be absent. Laboratory data show low or marginally raised levels of white blood cells and C-reactive protein, reflecting, respectively, the subacute or acute character of the infection. It is a self-limiting affection due to the adequate immune response of the patient. Evolution is generally good without antibiotherapy with a progressive spontaneous healing. A wait-and-see approach with close follow-up in the first weeks is the best therapeutic option.


2020 ◽  
Vol 14 (12) ◽  
pp. 1368-1373
Author(s):  
Yin Zhu ◽  
Yongping Chen ◽  
Yuewen Gong ◽  
Aijun Pan ◽  
Huijie Ding ◽  
...  

Introduction: Current pandemic of the coronavirus induced disease 2019 (COVID-19) presents an urgent issue to the world due to lack of vaccine and medication. Hydroxychloroquine (HCQ) has generated a lot of controversies whether it is effective in prevention and treatment of COVID-19. Current report presents a 63-year-old woman who has taken HCQ for many years but still infected by COVID-19. Case presentation: A patient with rheumatoid arthritis came to the clinic with fever and sore throat. The patient has been treated with 200 mg HCQ per day since 2016. Laboratory tests showed that the patient had lymphopenia, increased levels of high-sensitive C-reactive protein (hs-CRP) and serum Interleukin-6 (IL-6). Chest radiography showed that the patient had pneumonia. Throat swab test confirmed COVID-19 positive. On admission, she was treated with nebulized interferon alfa-2b, oral Lopinavir/Ritonavir, and ceftriaxone sodium for the COVID-19 in addition to HCQ. The patient stayed in hospital for 18 days, recovered from oxygen intake, and eventually discharged from hospital. Follow up investigation showed the patient developed antibody against COVID-19. Conclusions: Long-term application of HCQ could not prevent COVID-19 infection, but whether HCQ exerts benefit to alleviation of clinical symptoms and duration of hospital stays remains to be further investigated.


2019 ◽  
Vol 21 (1) ◽  
Author(s):  
Aleksey Mitev ◽  
Lisa Christ ◽  
Daria Feldmann ◽  
Moritz Binder ◽  
Kim Möller ◽  
...  

Abstract Background/purpose Elevated levels of C-reactive protein (CRP) in systemic sclerosis (SSc) have been linked to early inflammatory stages of the disease. This study has been designed to investigate CRP levels longitudinally in a cohort of SSc patients and to correlate these findings with comorbidities and disease characteristics. Methods In this retrospective study, patients with SSc treated at the outpatient clinic of the Department of Rheumatology and Clinical Immunology, University Medical Center Freiburg, were analyzed. Only patients with at least three consecutive visits and at least 1 year follow-up were included in this study. CRP serum levels were measured at every visit and categorized as positive if CRP concentrations were ≥ 5 mg/l. Subjects with elevated CRP levels at more than 80% of visits were defined as inflammatory SSc. The longitudinal CRP profiles were correlated with disease characteristics and comorbidities. Results A total of 1815 consecutive visits of 131 SSc patients were analyzed. Over the observed time span (7.6 (1.0–19.5) years), 18.3% (n = 24) of patients had continuously elevated CRP levels (inflammatory SSc), whereas in 29% (n = 38), CRP levels were always in the normal range. There was no association between disease duration and CRP levels at first visit. Inflammatory SSc was associated with male gender (p = 0.022), anti-Scl-70 antibodies (p = 0.009), diffuse cutaneous SSc (p = 0.036), pulmonary fibrosis (p < 0.001), rheumatoid arthritis (p = 0.007), and cardiac arrhythmia (p = 0.048). Moreover, patients with inflammatory SSc revealed higher modified Rodnan skin scores (p < 0.001); lower forced vital capacity (FVC) (p < 0.001), total lung capacity (p = 0.001), and diffusing capacity (p = 0.008); and faster decline of FVC per year (p = 0.007). Even treatment with cyclophosphamide (CYC) did not decrease CRP levels (p = 0.754). Conclusion Inflammatory SSc is characterized by a more severe phenotype, high morbidity, and a large proportion of male patients. Even treatment with CYC does not alter CRP levels in this subpopulation with a high unmet medical need.


2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Guangru Cao ◽  
JingCheng Rao ◽  
Yuqiang Cai ◽  
Chong Wang ◽  
Wenbo Liao ◽  
...  

The objective of this study was to investigate the treatment and prognosis of patients with spinal tuberculosis in Guizhou province. A total of 863 patients with spinal tuberculosis admitted to our hospital from 2006 to 2017 were included in this study. All patients underwent standardized quadruple antituberculosis treatment. Eighty patients were lost to follow-up due to a change of their contact information or noncompliance. A total of 783 patients completed the follow-up. The average follow-up period was 20.33 ± 8.77 months (range: 6 to 38 months). Among these patients, 145 patients underwent conservative treatment, while 638 patients underwent surgical treatment. All patients in the surgery group were treated with lesion removal, bone graft fusion, and internal fixation. Preoperative and postoperative standard quadruple antituberculosis treatment was administered. The clinical efficacy was evaluated according to erythrocyte sedimentation rate (ESR), c-reactive protein (CRP), visual analogue scale (VAS), Cobb angle correction, neurological functional recovery, and interbody fusion with bone graft and tuberculosis outcome. A total of 608 patients achieved clinical cure. The symptoms, physical signs, blood tests and imaging findings were improved in 143 patients. Twenty patients showed refractory clinical symptoms, and 12 patients had local tuberculosis recurrence. Conservative and surgical treatments are the mainstream treatments for spinal tuberculosis. According to the patients’ individual conditions, individualized treatments should be used to achieve good efficacy. Standardized antituberculosis treatment should be applied over the course of spinal tuberculosis.


2017 ◽  
Vol 4 (5) ◽  
pp. 1594 ◽  
Author(s):  
Uday Shankar Baluni ◽  
Tejas Mistry ◽  
Ankur Kothari ◽  
Tejas Patel

Background: Corrosive gastrointestinal tract injuries are a source of considerable morbidity all over the world and differ in their presentations.Methods: In the present work, study was done on 41 patients with history of acute corrosive injury with a period of 24 hours of ingestion and analysis on the parameters of age, sex, mode of ingestion, nature of corrosive, clinical symptoms. Further UGI scopy was also done within 24 to 48 hours of admissions.Results: With the study it was found that, the incidence of corrosive ingestion was reducing as the age was increasing and among all patients 27 were females and only 14 were males. The most common cause of corrosive ingestion was suicidal found in 30 cases and only 11 were accidental and most common nature of corrosive used was acid. 2 patients get expired during the initial resuscitation. On GI scopy, 18 have grade 1, 12 have grade 0, 4 have grade 2 and 7 have grade 3 according to Zargar classification. Initially 35 patients were given conservative treatment and 4 cases were treated surgically by feeding jejunostomy. On follow up 31 were advised for conservative management and 7 required surgical management, out of which esophageal dilatation was done in 2 cases, esophageactomy in 2 cases and gastrojejunostomy was done in cases and one case get expired.Conclusions: Corrosive ingestion patient are managed on the basis of UGI scopic grading.


Author(s):  
Eva Rodríguez ◽  
Belén Latzke ◽  
Milagros Sierra ◽  
Ana María Romera ◽  
Diego Siedel ◽  
...  

Abstract Background The value of myeloperoxidase and proteinase 3 antibodies titers in the assessment of renal disease activity and flare prediction in patients with ANCA-associated-vasculitis (AAV) is not well-known. Methods Retrospective study including 113 AVV patients with a renal biopsy-proven pauci-immune necrotizing glomerulonephritis from seven Spanish hospitals. The main inclusion criteria were assessment of MPO antibodies (MPOab) using multiplex flow immunoassay and PR3 antibodies (PR3ab) measurements using immunoassay chemiluminescence with an identical range of values for all participating centers. Results Serum MPOab, 3 ± 1.2 months before relapse, was higher in patients who relapsed (19.2 ± 12.2 vs 3.2 ± 5.1 AI, p &lt; 0.001). The discrimination value of MPOab 3 months before renal relapse had an AUC of 0.82 (95%CI 0.73-0.92; p &lt; 0.001). ΔMPOab (change in antibodies titration 6 months before relapse) was higher in patients who relapsed [8.3 ± 12 vs 0.9 ± 3.1 AI, p = 0.001) (AI; antibody index unit). The discrimination value of ΔMPO had an AUC of 0.76 (95%CI 0.63-0.88; p &lt; 0.001). The positive predictive value of renal relapse in PR3 patients is 100% and the negative predictive value of renal relapse in patients with PR3 positive titers is 57.1%. Serum PR3ab was higher in patients who relapsed 2.8 ± 1.4 months before relapse (58.6 ± 24.6 vs 2.0 ± 0.6 AI, p &lt; 0.001). Conclusions MPO antibody level monitorization using multiplex flow immunoassay and PR3 measurements using immunoassay chemiluminescence are useful and sensitive tools for the prediction of renal relapse in the follow-up of AAV patients with renal disease, and relevant surrogate markers of renal disease activity.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yifan Ren ◽  
Chuanxin Zhang ◽  
Xiaoqin Xu ◽  
Yu Yin

Abstract Background Kawasaki disease (KD) is the most common cause of acquired heart disease among children in developed countries, in which the resulting coronary artery (CA) abnormalities cause myocardial ischemia, infarction, and death. Prompt diagnosis was essential, and supplemental information should be used to assist the diagnosis when classical clinical criteria are incomplete. The elevated levels of serum transaminases in most KD patients are mild. Herein, a case of atypical KD child with severely elevated transaminase was reported. Case presentation A child with clinical manifestations of fever, high C-reactive protein (CRP) and severely elevated transaminases was reported. The treatment effect of antibiotic and liver-protecting drugs was not satisfactory. A bilateral diffuse dilation of the CA was detected on echocardiography on day 5 of the illness; thus, atypical KD was diagnosed. Elevated transaminases declined rapidly to normal after the treatment of intravenous immunoglobulin (IVIG). A 1-month follow-up revealed that CA returned to normal, and 2-month, 6-months, and 1-year follow-up revealed the child was in good general health. Conclusions This case highlighted that atypical KD clinical symptoms were diverse, and severely elevated transaminases might provide a clue to healthcare providers for the diagnosis and management of atypical KD.


1998 ◽  
Vol 39 (3) ◽  
pp. 315-321 ◽  
Author(s):  
H. B. Eggesbø ◽  
T. Jacobsen ◽  
E. Kolmannskog ◽  
D. Bay ◽  
K. Nygaard

Purpose: To compare the findings at US, CT and contrast enema (CE) with the clinical, biochemical, and surgical findings in patients with suspected acute left-sided colonic diverticulitis (ALCD) Material and Methods: The study comprised 32 consecutive patients hospitalized with clinically diagnosed ALCD. During the first 3 days of hospitalization, they were examined by US and CT of the pelvis and abdomen, and CE Results: of the 32 patients, 21 had ALCD according to the combined radiological, clinical, biochemical and surgical findings. Findings compatible with ALCD were verified by radiological examination alone in 12 patients (4 patients at US, 10 at CT, and 6 at CE). In 9 patients, only diverticula or tethering was found at either CE or CT, and the diagnoses were then supported by clinical and biochemical findings and clinical follow-up. The most common clinical symptoms and signs associated with ALCD were left lower quadrant pain, left-sided tenderness, and fever. Most patients showed elevations of the white blood-cell count, erythrocyte sedimentation rate, and C-reactive protein, but there was no statistical difference in the stages of severity of ALCD, or between ALCD and other abdominal disorders Conclusion: Radiological examination is valuable in confirming the diagnosis of ALCD. CT was the radiological modality that best demonstrated ALCD and revealed complications (e.g. pericolic abscesses) during the course of the illness


Author(s):  
Jia-Huei Yan ◽  
Ling-Sai Chang ◽  
Yi-Ju Lin ◽  
Mindy Ming-Huey Guo ◽  
Ying-Hsien Huang ◽  
...  

Background: Kawasaki disease (KD) is a form of vasculitis that primarily affects children under the age of 5 years old. Patients may be missed diagnosis when initial clinical symptoms do not fulfill the traditional criteria. We aimed to analyze factors that clinicians could use to differentiate febrile children suspected of KD. Method: We retrospectively enrolled a total of 83 febrile children who were initially suspected of KD, but they did not meet the American Heart Association (AHA) criteria for a diagnosis. However, some of these patients were diagnosed with KD during their second visit. We analyzed patients' characteristics, clinical symptoms, and laboratory data. Results: In total, 50 patients were enrolled in the study. Of those, ten patients were diagnosed with KD on their second visit (group 1), while the other 40 patients still did not fit a KD diagnosis (group 2). A patient with a neutrophil to lymphocyte ratio greater than 1.33 combined with a C-reactive protein more than 33 mg/L was more likely to have KD. Conclusion: Among patients suspected of KD that did not initially meet the criteria, clinicians should pay special attention to elevated neutrophil-to-lymphocyte ratios and CRP levels and closely follow up such patients.


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