Pub Date : 2024-03-22DOI: 10.4081/reumatismo.2024.1669
D Oliveira, A Martins, F Martins, M Rato, F Pinheiro, D Fonseca, C Vaz, E Mariz, L Costa
Spontaneous subcapsular and perirenal hemorrhage, known as Wunderlich syndrome (WS), is a rare clinical manifestation of polyarteritis nodosa (PAN). We report a case of a 48-year-old male with a history of recurrent episodes of leg muscle tenderness and dysesthesia, bilateral flank pain, painful nodular skin lesions in the lower limbs, weight loss, and difficult-to-control arterial hypertension. The abdominopelvic computed tomography angiography showed a large left perirenal hematoma, leading to the patient's admission to the intensive care unit. After the exclusion of infectious or neoplastic foci, the patient was diagnosed with PAN and started intravenous methylprednisolone pulses with a good response. Since WS is a rare initial clinical manifestation of PAN, an early diagnosis and aggressive treatment will significantly improve clinical outcomes.
自发性囊下和肾周出血,即 Wunderlich 综合征(WS),是结节性多动脉炎(PAN)的一种罕见临床表现。我们报告了一例 48 岁男性患者的病例,该患者有反复发作的腿部肌肉触痛和感觉障碍、双侧侧腹疼痛、下肢结节性皮肤疼痛性病变、体重减轻和难以控制的动脉高血压病史。腹盆腔计算机断层扫描血管造影显示左肾周巨大血肿,患者因此被送入重症监护室。在排除感染性或肿瘤性病灶后,患者被诊断为 PAN,开始静脉注射甲基强的松龙脉冲,反应良好。由于 WS 是 PAN 罕见的初始临床表现,早期诊断和积极治疗将显著改善临床预后。
{"title":"Wunderlich syndrome as a rare complication of polyarteritis nodosa: a case report.","authors":"D Oliveira, A Martins, F Martins, M Rato, F Pinheiro, D Fonseca, C Vaz, E Mariz, L Costa","doi":"10.4081/reumatismo.2024.1669","DOIUrl":"10.4081/reumatismo.2024.1669","url":null,"abstract":"<p><p>Spontaneous subcapsular and perirenal hemorrhage, known as Wunderlich syndrome (WS), is a rare clinical manifestation of polyarteritis nodosa (PAN). We report a case of a 48-year-old male with a history of recurrent episodes of leg muscle tenderness and dysesthesia, bilateral flank pain, painful nodular skin lesions in the lower limbs, weight loss, and difficult-to-control arterial hypertension. The abdominopelvic computed tomography angiography showed a large left perirenal hematoma, leading to the patient's admission to the intensive care unit. After the exclusion of infectious or neoplastic foci, the patient was diagnosed with PAN and started intravenous methylprednisolone pulses with a good response. Since WS is a rare initial clinical manifestation of PAN, an early diagnosis and aggressive treatment will significantly improve clinical outcomes.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"76 1","pages":""},"PeriodicalIF":1.4,"publicationDate":"2024-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1593
F I Gorial, N I Awadh, N W Shaheen, A Dheyaa, Z H Ali, N Wajdi
Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that affects multiple organs and causes inflammation, necrosis, and vasculitis in small blood vessels. Treatment for GPA involves achieving and maintaining remission. In recent studies, cyclophosphamide-based regimens have been linked to comorbidity hazards, including an increased risk of malignancies, especially hematological ones. Acute myeloid leukemia is the main hematologic malignancy that can complicate GPA. In this context, we report the case of a middle-aged woman with GPA who developed acute promyelocytic leukemia (APL) during maintenance with cyclophosphamide. She was treated with all-trans retinoic acid at 50 mg/day and arsenic trioxide at 10 mg/day, along with steroids. This case highlights the unique emergence of APL in a GPA patient during cyclophosphamide therapy. A single case has previously been reported on the development of APL in a patient with GPA while using azathioprine monotherapy for 2 years.
{"title":"Emergence of acute promyelocytic leukemia in a patient with granulomatosis with polyangiitis during treatment with cyclophosphamide: a rare case report.","authors":"F I Gorial, N I Awadh, N W Shaheen, A Dheyaa, Z H Ali, N Wajdi","doi":"10.4081/reumatismo.2023.1593","DOIUrl":"10.4081/reumatismo.2023.1593","url":null,"abstract":"<p><p>Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that affects multiple organs and causes inflammation, necrosis, and vasculitis in small blood vessels. Treatment for GPA involves achieving and maintaining remission. In recent studies, cyclophosphamide-based regimens have been linked to comorbidity hazards, including an increased risk of malignancies, especially hematological ones. Acute myeloid leukemia is the main hematologic malignancy that can complicate GPA. In this context, we report the case of a middle-aged woman with GPA who developed acute promyelocytic leukemia (APL) during maintenance with cyclophosphamide. She was treated with all-trans retinoic acid at 50 mg/day and arsenic trioxide at 10 mg/day, along with steroids. This case highlights the unique emergence of APL in a GPA patient during cyclophosphamide therapy. A single case has previously been reported on the development of APL in a patient with GPA while using azathioprine monotherapy for 2 years.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1598
N Sanghavi, B Ayesha
Anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is a B-cell-mediated autoimmune encephalitis with wide non-specific symptoms like acute-onset psychiatric or neurological ones mimicking various other conditions. A careful history and appropriate workup, including cerebrospinal fluid analysis for anti-NMDAR antibodies, imaging, and electroencephalogram, should be conducted, considering all differential diagnoses that can mimic its presentation. Combination therapy with high-dose steroids, plasma exchange, or immunoglobulin therapy has been shown to be more efficacious. In patients who fail first-line therapy, rituximab or cyclophosphamide should be considered. It is essential to rule out ovarian teratoma or other occult malignancies that can cause NMDARE, as removal of the tumor itself resolves this condition. Timely diagnosis and early intervention are necessary to avoid an untoward outcome.
抗 N-甲基-d-天冬氨酸受体脑炎(NMDARE)是一种由 B 细胞介导的自身免疫性脑炎,具有广泛的非特异性症状,如急性发作的精神或神经症状,可模拟各种其他疾病。应仔细询问病史并进行适当的检查,包括脑脊液抗 NMDAR 抗体分析、影像学检查和脑电图检查,同时考虑所有可能与该病表现相似的鉴别诊断。大剂量类固醇、血浆置换或免疫球蛋白疗法的联合治疗已被证明更为有效。对于一线治疗失败的患者,应考虑使用利妥昔单抗或环磷酰胺。必须排除卵巢畸胎瘤或其他可能导致 NMDARE 的隐匿性恶性肿瘤,因为切除肿瘤本身就能解决这种病症。及时诊断和早期干预是避免不良后果的必要条件。
{"title":"Anti-N-methyl-d-aspartate receptor encephalitis: mimicker of lupus and multiple sclerosis.","authors":"N Sanghavi, B Ayesha","doi":"10.4081/reumatismo.2023.1598","DOIUrl":"10.4081/reumatismo.2023.1598","url":null,"abstract":"<p><p>Anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is a B-cell-mediated autoimmune encephalitis with wide non-specific symptoms like acute-onset psychiatric or neurological ones mimicking various other conditions. A careful history and appropriate workup, including cerebrospinal fluid analysis for anti-NMDAR antibodies, imaging, and electroencephalogram, should be conducted, considering all differential diagnoses that can mimic its presentation. Combination therapy with high-dose steroids, plasma exchange, or immunoglobulin therapy has been shown to be more efficacious. In patients who fail first-line therapy, rituximab or cyclophosphamide should be considered. It is essential to rule out ovarian teratoma or other occult malignancies that can cause NMDARE, as removal of the tumor itself resolves this condition. Timely diagnosis and early intervention are necessary to avoid an untoward outcome.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808554","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1583
G Tamborrini, T Hügle, V Ricci, G Filippou
Objective: The prevalence of crystal arthropathies in the general population is rising. The purpose of this pictorial study is to describe the sonographic elements of the most prevalent crystal arthropathies by emphasizing particular sonographic findings using illustrative images and cases while considering technical details and common pitfalls.
Methods: Using established recommendations, specialists in the fields of sonography and crystal arthropathies agreed by consensus on the unique ultrasound signs associated with each of the conditions.
Results: Gout, calcium pyrophosphate deposition arthropathy, and hydroxyapatite arthropathy are the three most prevalent crystal arthropathies. Today's high-resolution sonography enables reliable evaluation of the underlying crystal deposits, post-inflammatory changes, and a precise description of joint inflammation.
Conclusions: High-prevalence crystal arthropathies are reliably detectable by ultrasound with current ultrasound equipment. It is necessary to have extensive ultrasound training, know specific sonographic findings, and understand all possible differential diagnoses for disorders affecting the musculoskeletal system.
{"title":"Ultrasound imaging in crystal arthropathies: a pictorial review.","authors":"G Tamborrini, T Hügle, V Ricci, G Filippou","doi":"10.4081/reumatismo.2023.1583","DOIUrl":"10.4081/reumatismo.2023.1583","url":null,"abstract":"<p><strong>Objective: </strong>The prevalence of crystal arthropathies in the general population is rising. The purpose of this pictorial study is to describe the sonographic elements of the most prevalent crystal arthropathies by emphasizing particular sonographic findings using illustrative images and cases while considering technical details and common pitfalls.</p><p><strong>Methods: </strong>Using established recommendations, specialists in the fields of sonography and crystal arthropathies agreed by consensus on the unique ultrasound signs associated with each of the conditions.</p><p><strong>Results: </strong>Gout, calcium pyrophosphate deposition arthropathy, and hydroxyapatite arthropathy are the three most prevalent crystal arthropathies. Today's high-resolution sonography enables reliable evaluation of the underlying crystal deposits, post-inflammatory changes, and a precise description of joint inflammation.</p><p><strong>Conclusions: </strong>High-prevalence crystal arthropathies are reliably detectable by ultrasound with current ultrasound equipment. It is necessary to have extensive ultrasound training, know specific sonographic findings, and understand all possible differential diagnoses for disorders affecting the musculoskeletal system.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808613","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1594
C Granjo Morais, A Martins, S Ganhão, F Aguiar, M Rodrigues, I Brito
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in pediatric patients. It is clinically characterized by fever flares lasting 3-7 days, reappearing every 2-8 weeks with a distinctive clockwork regularity. PFAPA generally begins before 5 years of age and usually ceases 3-5 years after onset. Recurrences may be observed in adolescence and adulthood in up to 20% of cases. The authors aim to describe a case of PFAPA recurrence in adolescence temporally associated with allergen-specific immunotherapy (ASIT). A 16-year-old female patient was referred to the rheumatology unit due to recurrent episodes of fever one month after initiating ASIT for allergic rhinitis. These episodes occurred every 4 weeks and lasted 3 days. During these episodes, she also presented with a sore throat, tonsillar exudates, and cervical lymphadenopathy. Abortive treatment with oral prednisolone was attempted in these episodes, with complete resolution of fever after a single dose. After reviewing her medical background, she had previously experienced febrile episodes accompanied by aphthous ulcers and tonsillar exudates occurring every 7-8 weeks from age 2-7. The etiopathogenesis of PFAPA remains uncertain. Environmental triggers, particularly those with immunomodulator effects, may interfere with the immune responses responsible for PFAPA occurrence, but the mechanisms are still unclear. The authors describe the first report of the reappearance of PFAPA flares, possibly due to ASIT. Further studies are needed to fully clarify if ASIT constitutes a true environmental trigger of PFAPA.
{"title":"Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis recurrence temporally associated with allergen-specific immunotherapy in a female adolescent: a case report.","authors":"C Granjo Morais, A Martins, S Ganhão, F Aguiar, M Rodrigues, I Brito","doi":"10.4081/reumatismo.2023.1594","DOIUrl":"10.4081/reumatismo.2023.1594","url":null,"abstract":"<p><p>Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in pediatric patients. It is clinically characterized by fever flares lasting 3-7 days, reappearing every 2-8 weeks with a distinctive clockwork regularity. PFAPA generally begins before 5 years of age and usually ceases 3-5 years after onset. Recurrences may be observed in adolescence and adulthood in up to 20% of cases. The authors aim to describe a case of PFAPA recurrence in adolescence temporally associated with allergen-specific immunotherapy (ASIT). A 16-year-old female patient was referred to the rheumatology unit due to recurrent episodes of fever one month after initiating ASIT for allergic rhinitis. These episodes occurred every 4 weeks and lasted 3 days. During these episodes, she also presented with a sore throat, tonsillar exudates, and cervical lymphadenopathy. Abortive treatment with oral prednisolone was attempted in these episodes, with complete resolution of fever after a single dose. After reviewing her medical background, she had previously experienced febrile episodes accompanied by aphthous ulcers and tonsillar exudates occurring every 7-8 weeks from age 2-7. The etiopathogenesis of PFAPA remains uncertain. Environmental triggers, particularly those with immunomodulator effects, may interfere with the immune responses responsible for PFAPA occurrence, but the mechanisms are still unclear. The authors describe the first report of the reappearance of PFAPA flares, possibly due to ASIT. Further studies are needed to fully clarify if ASIT constitutes a true environmental trigger of PFAPA.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1602
C Gioia, A Picchianti Diamanti, R Perricone, M S Chimenti, A Afeltra, L Navarini, A Migliore, U Massafra, V Bruzzese, P Scolieri, C Meschini, M Paroli, R Caccavale, P Scapato, R Scrivo, F Conti, B Laganà, M Di Franco
Objective: To compare etanercept and adalimumab biosimilars (SB4 and ABP501) and respective bioriginators in terms of safety and efficacy in a real-life contest.
Methods: We consequently enrolled patients affected by rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis, treated with SB4, and ABP501, or with corresponding originators, belonging to the main biological prescribing centers in the Lazio region (Italy), from 2017 to 2020. Data were collected at recruitment and after 4, 8, 12, and 24 months of therapy.
Results: The multicenter cohort was composed by 455 patients treated with biosimilars [SB4/ABP501 276/179; female/male 307/146; biologic disease-modifying anti-rheumatic drug (b-DMARD) naïve 56%, median age/ interquartile range 55/46-65 years] and 436 treated with originators (etanercept/adalimumab 186/259, female/ male 279/157, b-DMARD naïve 67,2%, median age/interquartile range 53/43-62 years). No differences were found about safety, but the biosimilar group presented more discontinuations due to inefficacy (p<0.001). Female gender, being a smoker, and being b-DMARD naïve were predictive factors of reduced drug survival (p=0.05, p=0.046, p=0.001 respectively). The retention rate at 24 months was 81.1% for bioriginators and 76.5% for biosimilars (median retention time of 20.7 and 18.9 months, respectively) (p=0.002). Patients with remission/low disease activity achievement at 4 months showed a cumulative survival of 90% to biosimilar therapy until 24 months (p=0.001); early adverse reactions instead represented a cause of subsequent drug discontinuation (p=0.001).
Conclusions: Real-life data demonstrated a similar safety profile between biosimilars and originators, but a reduced biosimilar retention rate at 24 months. Biosimilars could be considered a valid, safe, and less expensive alternative to originators, allowing access to treatments for a wider patient population.
{"title":"Anti-tumor necrosis factor α: originators <i>versus</i> biosimilars, comparison in clinical response assessment in a multicenter cohort of patients with inflammatory arthropathies.","authors":"C Gioia, A Picchianti Diamanti, R Perricone, M S Chimenti, A Afeltra, L Navarini, A Migliore, U Massafra, V Bruzzese, P Scolieri, C Meschini, M Paroli, R Caccavale, P Scapato, R Scrivo, F Conti, B Laganà, M Di Franco","doi":"10.4081/reumatismo.2023.1602","DOIUrl":"10.4081/reumatismo.2023.1602","url":null,"abstract":"<p><strong>Objective: </strong>To compare etanercept and adalimumab biosimilars (SB4 and ABP501) and respective bioriginators in terms of safety and efficacy in a real-life contest.</p><p><strong>Methods: </strong>We consequently enrolled patients affected by rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis, treated with SB4, and ABP501, or with corresponding originators, belonging to the main biological prescribing centers in the Lazio region (Italy), from 2017 to 2020. Data were collected at recruitment and after 4, 8, 12, and 24 months of therapy.</p><p><strong>Results: </strong>The multicenter cohort was composed by 455 patients treated with biosimilars [SB4/ABP501 276/179; female/male 307/146; biologic disease-modifying anti-rheumatic drug (b-DMARD) naïve 56%, median age/ interquartile range 55/46-65 years] and 436 treated with originators (etanercept/adalimumab 186/259, female/ male 279/157, b-DMARD naïve 67,2%, median age/interquartile range 53/43-62 years). No differences were found about safety, but the biosimilar group presented more discontinuations due to inefficacy (p<0.001). Female gender, being a smoker, and being b-DMARD naïve were predictive factors of reduced drug survival (p=0.05, p=0.046, p=0.001 respectively). The retention rate at 24 months was 81.1% for bioriginators and 76.5% for biosimilars (median retention time of 20.7 and 18.9 months, respectively) (p=0.002). Patients with remission/low disease activity achievement at 4 months showed a cumulative survival of 90% to biosimilar therapy until 24 months (p=0.001); early adverse reactions instead represented a cause of subsequent drug discontinuation (p=0.001).</p><p><strong>Conclusions: </strong>Real-life data demonstrated a similar safety profile between biosimilars and originators, but a reduced biosimilar retention rate at 24 months. Biosimilars could be considered a valid, safe, and less expensive alternative to originators, allowing access to treatments for a wider patient population.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1543
M B Ates, S Karup, S Ugurlu
Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited autoinflammatory disease characterized by systemic inflammation and immunodeficiency. Infliximab proved to be favorable in the treatment of this condition. This case report is concerned with a DADA2 deficient patient treated with infliximab. This is a rare case of DADA2 in a 32-year-old female patient. The patient was admitted with a clinical presentation of erythema, ulcers, and pruritus on both legs and ankles, accompanied by red ulcerative oral lesions, fatigue, malaise, and dizziness. The patient's genetic analysis was positive for DADA2. Treatment based on TNF-α inhibition was highly effective for this patient. We used laboratory testing and punch biopsy as differential diagnostic tools, where antinuclear antibody positivity, high prolactin levels, and high serum C-reactive protein were observed. The punch biopsy revealed both orthohyperkeratosis and parahyperkeratosis of the dermis, diffuse core fragments, plasma in the stratum corneum, and hypergranulous acanthosis. DADA2 treatment is centered on tumor necrosis factor α suppression. Although high-dose systemic glucocorticoids can reduce inflammation in the initial stages of the disease, most patients have a resistant or relapsing response to tapering attempts. The prevalence of undiagnosed cases of autoinflammatory diseases is anticipated to diminish with the growing awareness of them.
{"title":"Infliximab as successful treatment option in a case of adenosine deaminase 2 deficiency.","authors":"M B Ates, S Karup, S Ugurlu","doi":"10.4081/reumatismo.2023.1543","DOIUrl":"10.4081/reumatismo.2023.1543","url":null,"abstract":"<p><p>Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited autoinflammatory disease characterized by systemic inflammation and immunodeficiency. Infliximab proved to be favorable in the treatment of this condition. This case report is concerned with a DADA2 deficient patient treated with infliximab. This is a rare case of DADA2 in a 32-year-old female patient. The patient was admitted with a clinical presentation of erythema, ulcers, and pruritus on both legs and ankles, accompanied by red ulcerative oral lesions, fatigue, malaise, and dizziness. The patient's genetic analysis was positive for DADA2. Treatment based on TNF-α inhibition was highly effective for this patient. We used laboratory testing and punch biopsy as differential diagnostic tools, where antinuclear antibody positivity, high prolactin levels, and high serum C-reactive protein were observed. The punch biopsy revealed both orthohyperkeratosis and parahyperkeratosis of the dermis, diffuse core fragments, plasma in the stratum corneum, and hypergranulous acanthosis. DADA2 treatment is centered on tumor necrosis factor α suppression. Although high-dose systemic glucocorticoids can reduce inflammation in the initial stages of the disease, most patients have a resistant or relapsing response to tapering attempts. The prevalence of undiagnosed cases of autoinflammatory diseases is anticipated to diminish with the growing awareness of them.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1588
B Cremonezi Lammoglia, L De Aguiar Trevise, T Paslar Leal, M Pereira Lopes Vieira Pinto, G Hasselmann, N Salles Rosa Neto
We report the case of a 54-year-old woman with antineutrophilic cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis presenting with mononeuritis multiplex, intestinal hemorrhage, cardiomyopathy, fever, and worsening asthma symptoms. She was initially treated with steroids and cyclophosphamide but eventually required rituximab to control a vasculitis flare. However, her asthmatic symptoms did not improve, despite attaining vasculitis remission. Symptoms abated only after the treatment transition to mepolizumab. After a 1-year interval, there were no further episodes of asthma exacerbation and no requirement for systemic steroid therapy. This report reinforces the use of rituximab for induction and maintenance of remission in patients with eosinophilic granulomatosis with polyangiitis and predominant vasculitic manifestations, whereas mepolizumab demonstrated better control of the persistent eosinophilic manifestations, ensuing sustained remission and improved quality of life.
{"title":"Eosinophilic granulomatosis with polyangiitis: sequential use of mepolizumab following rituximab for inadequate asthma control despite vasculitis remission.","authors":"B Cremonezi Lammoglia, L De Aguiar Trevise, T Paslar Leal, M Pereira Lopes Vieira Pinto, G Hasselmann, N Salles Rosa Neto","doi":"10.4081/reumatismo.2023.1588","DOIUrl":"10.4081/reumatismo.2023.1588","url":null,"abstract":"<p><p>We report the case of a 54-year-old woman with antineutrophilic cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis presenting with mononeuritis multiplex, intestinal hemorrhage, cardiomyopathy, fever, and worsening asthma symptoms. She was initially treated with steroids and cyclophosphamide but eventually required rituximab to control a vasculitis flare. However, her asthmatic symptoms did not improve, despite attaining vasculitis remission. Symptoms abated only after the treatment transition to mepolizumab. After a 1-year interval, there were no further episodes of asthma exacerbation and no requirement for systemic steroid therapy. This report reinforces the use of rituximab for induction and maintenance of remission in patients with eosinophilic granulomatosis with polyangiitis and predominant vasculitic manifestations, whereas mepolizumab demonstrated better control of the persistent eosinophilic manifestations, ensuing sustained remission and improved quality of life.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1565
H Parente, M Pontes Ferreira, C Soares, F Guimarães, S Azevedo, D Santos-Faria, J Tavares-Costa, D Peixoto, C Afonso, D Roriz, F Teixeira
Lumbar pain is a very common symptom that derives from benign musculoskeletal conditions, rheumatic inflammatory diseases, neoplasms, and referred and/or nociplastic pain. A 70-year-old man with psoriatic arthritis presented with early-onset lumbosacral pain without evident red flags. Symptomatic treatment was unhelpful. Radiographic imaging showed subtle signs of a disease that could easily be missed. Magnetic resonance imaging revealed a massive prostatic malignancy with bone (sacral and iliopubic) metastasis. Awareness must be given not to disregard every lumbar pain as part of the preexisting rheumatic inflammatory disease (spondyloarthropathy in this case) or a common muscle/ligament/articular disarrangement. Persistence of pain, albeit not inflam-matory nor sharp in nature, despite adequate treatment might be just as important as an acute red flag and requires proper follow-up.
{"title":"Lumbosacral pain in a patient with psoriatic arthritis: when the rheumatic disease is innocent.","authors":"H Parente, M Pontes Ferreira, C Soares, F Guimarães, S Azevedo, D Santos-Faria, J Tavares-Costa, D Peixoto, C Afonso, D Roriz, F Teixeira","doi":"10.4081/reumatismo.2023.1565","DOIUrl":"10.4081/reumatismo.2023.1565","url":null,"abstract":"<p><p>Lumbar pain is a very common symptom that derives from benign musculoskeletal conditions, rheumatic inflammatory diseases, neoplasms, and referred and/or nociplastic pain. A 70-year-old man with psoriatic arthritis presented with early-onset lumbosacral pain without evident red flags. Symptomatic treatment was unhelpful. Radiographic imaging showed subtle signs of a disease that could easily be missed. Magnetic resonance imaging revealed a massive prostatic malignancy with bone (sacral and iliopubic) metastasis. Awareness must be given not to disregard every lumbar pain as part of the preexisting rheumatic inflammatory disease (spondyloarthropathy in this case) or a common muscle/ligament/articular disarrangement. Persistence of pain, albeit not inflam-matory nor sharp in nature, despite adequate treatment might be just as important as an acute red flag and requires proper follow-up.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-12-19DOI: 10.4081/reumatismo.2023.1627
F R Spinelli, F Conti, R Caporali, F Iannone, F Cacciapaglia, On Behalf Of The Steering Committee Of The Italian Society Of Rheumatology
Following the restrictions on the reimbursability of Janus kinase inhibitors introduced by the Italian Medicines Agency, the Italian Society of Rheumatology has drafted this document to shed light on the clinical conditions and reimbursability criteria set out in the prescription forms.
{"title":"Janus kinase inhibitors: between prescription authorization and reimbursability.","authors":"F R Spinelli, F Conti, R Caporali, F Iannone, F Cacciapaglia, On Behalf Of The Steering Committee Of The Italian Society Of Rheumatology","doi":"10.4081/reumatismo.2023.1627","DOIUrl":"10.4081/reumatismo.2023.1627","url":null,"abstract":"<p><p>Following the restrictions on the reimbursability of Janus kinase inhibitors introduced by the Italian Medicines Agency, the Italian Society of Rheumatology has drafted this document to shed light on the clinical conditions and reimbursability criteria set out in the prescription forms.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":"75 4","pages":""},"PeriodicalIF":1.4,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138808593","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}