scholarly journals Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Christelle Darrieutort-Laffite ◽  
Catherine Ansquer ◽  
Hélène Aubert ◽  
Françoise Kraeber-Bodéré ◽  
Agathe Masseau ◽  
...  

Abstract Objective To report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS). Methods A retrospective cohort study of patients with SchS followed between 2000 and 2020. Inclusion criteria included a diagnosis of SchS (Strasbourg criteria). All available bone scans were reviewed and scored according to the intensity and number of pathological sites. The scintigraphic score was compared with the clinical activity score, CRP level, and treatments. Results Twenty-five patients were included. Median age at diagnosis was 68 years. Eighty patients (72%) had SchS-related rheumatic pain. Most patients had a long-standing isolated rash before constitutional and/or rheumatic symptoms appeared. The monoclonal component level was usually very low (IgMκ in 22/25). Rheumatic pain predominated around the knees. Bone scans revealed abnormal tracer uptake in 15/18 (85%). The scintigraphic score correlated with clinical activity (r = 0.4, p < 0.02) and CRP level (r = 0.47, p < 0.01). The scintigraphic score was lower in patients receiving corticosteroids or IL1Ra (interleukin 1 receptor antagonist) than in untreated patients (median scores:2, 0, and 13, respectively; p < 0.05). Two patients developed Waldenström macroglobulinemia. Of the 22 surviving patients, median age at follow-up was 76 years. IL1Ra was used in 13 patients, with dramatic efficacy on both symptoms and bone scan features. Conclusions Rheumatic manifestations are very prevalent in SchS. However, bone pain can be misleading and contribute to misdiagnosis. Bone scan abnormalities are very prevalent and correlate with disease activity and treatments. IL1-Ra has a dramatic and durable efficacy but may not be required in every patient early on.

PEDIATRICS ◽  
1987 ◽  
Vol 79 (4) ◽  
pp. 587-592
Author(s):  
Deborah C. ter Meulen ◽  
Massoud Majd

A retrospective analysis of bone scans of 381 children with unexplained skeletal pain was made. Of these, findings are reported on 358 for whom there were sufficient clinical data. The bone scan results suggested trauma as the cause of pain in 43 patients, inflammatory disease in 73 patients, and neoplasia in ten patients. There was only one false-positive bone scan. Normal findings were obtained from 227 patients, in whom no significant skeletal disease was detected on follow-up, except for juvenile rheumatoid arthritis in 23 patients. Bone scintigraphy is, therefore, an important, noninvasive diagnostic test for evaluating children with obscure bone or joint pain. We recommend that this test be performed early in the evaluation of these children to arrive at the diagnosis expeditiously and with minimal patient discomfort and morbidity.


1999 ◽  
Vol 38 (02) ◽  
pp. 38-42
Author(s):  
E. Kaiserling ◽  
T. Klingebiel ◽  
U. Feine ◽  
D. Niethammer ◽  
P. Reuland

Summary Aim: Influenced by the incorrect diagnosis of a bone metastasis caused by bone necrosis we evaluated reasons and frequency of bone necrosis in patients referred for bone scanning in follow-up of tumors. Methods: Bone scans performed within two years on patients with primary bone tumors or tumors metastatic to bone were reviewed in respect to the final diagnosis bone necrosis. Results: We found the cases of three young patients who presented the appearance of hot spots on bone scintigrams which were finally diagnosed as bone necrosis. In two cases the diagnosis was based on histological findings, in one case the diagnosis was made evident by follow-up. All the three patients had been treated by chemotherapy and presented no other reason for the development of bone necrosis. Enhanced tracer uptake in all sites decreased within eight weeks up to two years without therapy. Conclusion: Single and multiple hot spots after chemotherapy may be originated by bone necrosis but mimikry metastases.


Author(s):  
А. Рыжков ◽  
A. Ryzhkov ◽  
Л. Яковлева ◽  
L. Yakovleva ◽  
А. Крылов ◽  
...  

Purpose: To evaluate the effectiveness of SPECT/CT for the assessment of graft viability following revascularized bone grafts in patients with mandibular (18) and maxilla (1) reconstruction. Material and methods: We investigated 19 patients with revascularized grafts from the fibula (16 patients), iliac crest (2 patients) and shoulder blade graft (1). For the follow up of all these patients, 99mTc-MDP bone scintigraphy and SPECT/CT was performed between 4–5 days postoperatively. The evaluation of the grafts was based on a comparison of tracer uptake between graft and the cranium. Results: 17 of 19 grafts had an uncomplicated clinical course. Complications in the graft occurred in two patients. In the analysis of planar scintigrams with uncomplicated healing were showed correct assessment in 10 of 17 patients and in 1of 2 patients with signs of necrosis of graft fragments. SPECT/CT was performed in addition to planar imaging. The increased uptake grafts were showed with SPECT/CT in all patients with uncomplicated clinical course. In the failed 2 grafts, decreased uptake was observed in both patients. In the analysis of SPECT/CT images the correct results were showed in all clinical cases. Conclusion: SPECT/CT performed within 6 days after the mandibular reconstruction is a useful tool to monitor the viability and early complications of revascularized mandibular and maxilla bone grafts. SPECT/CT is also recommended to interpretation of the bone scans and to precise assessment of graft viability.


2016 ◽  
Vol 34 (2_suppl) ◽  
pp. 226-226
Author(s):  
Aseem Anand ◽  
Michael J. Morris ◽  
Steven M. Larson ◽  
Mattias Ohlsson ◽  
Andreas Joseffson ◽  
...  

226 Background: The automated BSI (auto-BSI) is a computerized means of quantitatively expressing tumor burden in bone. Following the analytical validation of auto-BSI, we are now clinically qualifying the tool as a response biomarker in mCRPC. In the present study, we assess the association of auto-BSI and that of PSA with overall survival (OS) in mCRPC patients (pts) being treated with enzalutamide. Methods: All mCRPC pts who initiated enzalutamide treatment at Skåne Hospital, Sweden and at Rigshospitalet, Denmark, with a minimum of one year follow-up, were eligible for the study. Eligible mCRPC pts with baseline (BL) bone scan available before starting enzalutamide were enrolled in the study. PSA, hemoglobin (Hgb) and alkaline phosphatase (ALP) were obtained at BL. Treatment follow-up PSA and auto-BSI were obtained at week-12. auto-BSI was obtained using the analytically validated EXIN boneBSIversion 2.0. Cox regression and concordance index (c-index) were used to evaluate the association with OS. Results: 80 mCRPC pts with BL bone scans were enrolled in the study. Treatment follow-up bone scans were available from 62 pts. Univariate analysis demonstrated that auto-BSI, PSA, ALP and Hgb at BL were associated with OS (p < 0.0001). In the multivariate analysis, only auto-BSI and HgB were significantly associated with OS. The auto-BSI showed the highest c-index in prediction of OS (c-index 0.72, SE 0.03). Adding auto-BSI to the BL covariate model significantly improved the discrimination from c-index 0.67 to 0.72 (p < 0.05). At treatment follow-up, the univariate analysis of the change in auto-BSI and the percent change in PSA were strongly associated with OS with c-index 0.76 and 0.72 (SE 0.05), respectively. In bivariate analysis, the change in auto-BSI remained strongly associated with OS (p < 0.0001) whereas the percent change in PSA appeared to be less so (p = 0.07). Conclusions: Auto-BSI and its change demonstrated an independent and a strong association with OS in mCRPC pts being treated with enzalutamide. The study serve as a foundation for prospective validation of auto-BSI as an imaging biomarker indicative of efficacy to enzalutamide.


2016 ◽  
Vol 34 (2_suppl) ◽  
pp. 305-305 ◽  
Author(s):  
Yangmin M. Ning ◽  
Clara Chen ◽  
Virginia Ellen Maher ◽  
James Xunhai Xu ◽  
Geoffrey Kim ◽  
...  

305 Background: To differentiate between bone metastasis progression vs. Tc99m scan “flare” in new lesions on early bone scans ( ≤ 12 wks), ≥ 2 additional lesions on a confirmatory scan (6 wks later) are proposed. This reduces the risk of misreading scan “flare” as progression in responding patients (pts). Independent central review (ICR) of scans from placebo (PLC)-controlled trials can help evaluate the role of confirmatory scans as PLC should neither delay progression nor elicit scan “flare”. Methods: The ICR datasets from a randomized PLC-controlled trial of enzalutamide (ENZ) in pts with chemo-naïve mCRPC were examined. Pts with ≥ 2 new lesions on Week 9 bone scans who underwent confirmatory scans were analyzed. Scan “flare” was defined as unconfirmed progression associated with responses in PSA ( ≥ 50% decline). Results: Summarized in the table. Confirmed progression on Week 9 bone scans occurred more in pts on PLC than in pts on ENZ (57% vs. 14%). In pts with unconfirmed progression, scan “flare” occurred in 80% of pts on ENZ. Of the pts with unconfirmed progression who had PSA progression, nearly 60% progressed on follow-up scans. Conclusions: The findings from this large PLC-controlled trial provide strong evidence for performing confirmatory bone scans to verify tumor progression in new lesions on early bone scans in mCRPC. For pts with unconfirmed progression, early PSA progression appears associated with progression on follow-up scans. [Table: see text]


1992 ◽  
Vol 31 (06) ◽  
pp. 239-241 ◽  
Author(s):  
S. A. Qutaishat ◽  
A. Y. Al Hindawi

Summary1725 bone scans, done for evaluation of metastatic involvement from malignant neoplasms, were reviewed. The fraction of positive scans (multiple lesions in the bone scan) was 34% of the patients with different malignancies. In 251 patients (12%) a single bone lesion was detected and 114 of these patients were followed-up and re-scanned once or twice within 1 to 4 years. 39 of the 93 patients with solitary bone lesion who on follow-up developed multiple lesions had carcinoma of the breast as the primary.


2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Maaman Bashir ◽  
Brittany Bettendorf ◽  
Richard Hariman

Background. Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra. Objectives. To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. Cases. We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria. Both were treated with anakinra with remarkable response to therapy. Conclusion. Schnitzler syndrome is a rare and underdiagnosed disorder. High suspicion should be maintained in patients with chronic urticaria-like dermatoses, intermittent fevers, and arthralgias. A serum protein electrophoresis and immunofixation should be performed in these patients. The diagnosis is important to recognize as Schnitzler syndrome is associated with malignancy. A lymphoproliferative disorder develops in about 20% of patients at an average of 7.6 years after onset of symptoms. Thus, patients warrant long-term follow-up. IL-1 inhibitors are extremely effective in relieving symptoms and are considered first line therapy.


2019 ◽  
Vol 30 (5) ◽  
pp. 641-648
Author(s):  
Jong Ho Noh ◽  
Jae Young Lee ◽  
Sunwook Hwang ◽  
Kee Haeng Lee

Objective: To evaluate the predicting value of 99mTc-hydroxydiphosphonate (HDP) pinhole bone scan in development of osteonecrosis of femoral head (ONFH) in patients with femoral neck fracture after cannulated screw fixation. Methods: Pinhole bone scan of patients with metallically fixed femoral neck fracture from 2001 to 2015 were retrospectively reviewed. Initial pinhole bone scan was obtained within 2–3 weeks after surgery. Findings of initial pinhole bone scan were divided in to 4 groups. Group CU included cold defect in affected femoral head, group HU with no cold defect. Group PP with increased uptake along the inserted screws and group PN with no increased uptake along the inserted screws. More than 6 months of follow-up with pinhole bone scan and clinico-radiological evidence for ONFH was reviewed. Results: 72 patients (mean age 54.01 years, male 22, female 50) were included. 19 patients were in group CU, 53 in group HU. 60 patients were in group PP, 12 in group PN. During the follow-up, 13 patients were diagnosed as ONFH. 9 (47.36%) patients in group CU developed ONFH and 4 (7.5%) in group HU. 4 (6.66%) patients in group PP developed ONFH and 9 (75%) in group PN. Conclusions: To predict ONFH of femoral head followed by neck fracture, many imaging techniques with variable results were known. In this study, cold defect in early postoperative pinhole bone scans could predict ONFH, and loss of increased uptake along screw inserted site could be a strong indicative sign of ONFH. Further evaluation with a larger population is necessary.


1997 ◽  
Vol 12 (4) ◽  
pp. 158-161 ◽  
Author(s):  
S. Modoni ◽  
E. Calò ◽  
G. Nardella ◽  
G. Ritrovato ◽  
V. Frusciante

The authors evaluate the role of prostate specific antigen (PSA) and bone scintigraphy in the follow-up of radical prostatectomy-treated and radiotherapy-treated patients. 784 patients were evaluated by simultaneous PSA assay and bone scans. The correlation between PSA levels and extension of bone metastases was good. The frequency of extraskeletal metastases was low: only 13 patients had soft tissue metastases without bone involvement and 33/138 patients with bone metastases had also extraskeletal metastases. The results underline the importance of PSA and the possibility to omit bone scan when the PSA level is below 8 ng/ml in patients who did not undergo anti-androgenic treatments.


2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Kensaku Abe ◽  
Norio Yamamoto ◽  
Katsuhiro Hayashi ◽  
Akihiko Takeuchi ◽  
Shinji Miwa ◽  
...  

Abstract Background Intramedullary osteosclerosis (IMOS) is a rare condition without specific radiological findings except for the osteosclerotic lesion and is not associated with family history and infection, trauma, or systemic illness. Although the diagnosis of IMOS is confirmed after excluding other osteosclerotic lesions, IMOS is not well known because of its rarity and no specific feature. Therefore, these situations might result in delayed diagnosis. Hence, this case report aimed to investigate three cases of IMOS and discuss imaging findings and clinical outcomes. Case presentation All three cases were examined between 2015 and 2019. The location of osteosclerotic lesions were femoral diaphyses in the 60-year-old man (Case 1) and 41-year-old woman (Case 2) and tibial diaphysis in the 44-year-old woman (Case 3). All cases complained of severe pain and showed massive diaphyseal osteosclerotic lesions in plain radiograms and computed tomography (CT) scans. Cases 2 and 3 were examined using the triphasic bone scan, and a fusiform-shaped intense area of the tracer uptake on delayed bone image was detected in both cases without (Case 2) or slightly increased vascularity (Case 3) on the blood pool image, which was reported as a specific finding of IMOS. Open biopsy was performed in all cases, and histologic section showed trabecular bone sclerosis with hypocellular fibrous tissues, finally diagnosed as IMOS. The pain was sharply improved after biopsy and kept at the latest follow-up periods (34, 33, and 6 months in Cases 1, 2, and 3, respectively). Conclusions Massive sclerotic lesions with severe pain in the diaphyseal region of long bones should be considered as IMOS to avoid the delayed diagnosis, although other sclerotic bony lesions should be carefully excluded. Triphasic bone scan with a fusiform-shaped intense area of tracer uptake on delayed bone image and without or slightly increased vascularity on the blood pool image will help confirm IMOS. The role of open biopsy was to confirm the diagnosis of IMOS and to give the severe pain relief immediately in the three cases, although more cases and long-term follow-up are necessary.


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