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BMC Nephrology Jun 2024Sarcoidosis is a systemic disease that can affect multiple organs. While pulmonary sarcoidosis is most commonly observed, renal sarcoidosis occurs less frequently. We...
BACKGROUND
Sarcoidosis is a systemic disease that can affect multiple organs. While pulmonary sarcoidosis is most commonly observed, renal sarcoidosis occurs less frequently. We herein report a case of sarcoidosis with an exceptionally rare distribution including renal lesions.
CASE PRESENTATION
A 51-year-old Japanese female was referred because of bilateral parotid swelling and renal dysfunction. Computed tomography scan showed the swelling of bilateral kidneys, parotid glands, and uterus. Ga scintigraphy also showed remarkable accumulation in these organs. Renal biopsy and cytological evaluations of parotid gland and uterus were performed and she was diagnosed as sarcoidosis of these organs. Treatment was initiated with prednisolone 40 mg/day and then renal dysfunction subsequently improved. In addition, the swelling of parotid glands and uterus improved and Ga accumulation in each organ had disappeared.
CONCLUSION
This is a first case of renal sarcoidosis complicated by parotid glands and uterus lesions. Pathological findings and the reactivity observed in Ga scintigraphy indicated the presence of lesions in these organs.
Topics: Humans; Female; Middle Aged; Sarcoidosis; Kidney Diseases; Parotid Gland; Uterine Diseases; Prednisolone; Parotid Diseases; Radionuclide Imaging; Tomography, X-Ray Computed
PubMed: 38890580
DOI: 10.1186/s12882-024-03635-6 -
The Lancet. Haematology Jun 2024A standard of care and optimal duration of therapy have not been established for patients with multiply relapsed or refractory follicular lymphoma. The aim of this study...
BACKGROUND
A standard of care and optimal duration of therapy have not been established for patients with multiply relapsed or refractory follicular lymphoma. The aim of this study was to evaluate epcoritamab, a novel CD3 × CD20 bispecific antibody, in the third-line and later setting of follicular lymphoma.
METHODS
EPCORE NHL-1 is a multicohort, single-arm, phase 1-2 trial conducted at 88 sites across 15 countries. Here, we report the primary analysis of patients with relapsed or refractory follicular lymphoma in the phase 2 part of the trial, which included the pivotal (dose expansion) cohort and the cycle 1 optimisation cohort. Eligible patients were aged 18 years or older, had relapsed or refractory CD20 follicular lymphoma (grade 1-3A), an Eastern Cooperative Oncology Group performance status of up to 2, and had received at least two previous lines of therapy (including an anti-CD20 monoclonal antibody and an alkylating agent or lenalidomide). Patients were treated with subcutaneous epcoritamab 48 mg in 28-day cycles: weekly in cycles 1-3, biweekly in cycles 4-9, and every 4 weeks until disease progression or unacceptable toxicity. To mitigate the risk and severity of cytokine release syndrome, in the pivotal cohort, cycle 1 consisted of a step-up dosing regimen of a 0·16-mg priming dose on day 1 and a 0·80-mg intermediate dose on day 8, followed by subsequent 48-mg full doses and prophylactic prednisolone 100 mg; in the cycle 1 optimisation cohort, a second intermediate dose of 3 mg on day 15, adequate hydration, and prophylactic dexamethasone 15 mg were evaluated during cycle 1 to further reduce risk and severity of cytokine release syndrome. Primary endpoints were independently reviewed overall response rate for the pivotal cohort and the proportion of patients with grade 2 or worse and any-grade cytokine release syndrome for the cycle 1 optimisation cohort. Analyses were done in all enrolled patients who had received at least one dose of epcoritamab. This study is registered with ClinicalTrials.gov, NCT03625037, and is ongoing.
FINDINGS
Between June 19, 2020, and April 21, 2023, 128 patients (median age 65 years [IQR 55-72]; 49 [38%] female and 79 [62%] male) were enrolled and treated in the pivotal cohort (median follow-up 17·4 months [IQR 9·1-20·9]). The overall response rate was 82·0% (105 of 128 patients; 95% CI 74·3-88·3), with a complete response rate of 62·5% (80 of 128; 95% CI 53·5-70·9). The most common grade 3-4 treatment-emergent adverse event was neutropenia in 32 (25%) of 128 patients. Grade 1-2 cytokine release syndrome was reported in 83 (65%) of 128 patients; grade 3 cytokine release syndrome was reported in two (2%). Immune effector cell-associated neurotoxicity syndrome was reported in eight (6%) of 128 patients (five [4%] grade 1; three [2%] grade 2). Between Oct 25, 2022, and Jan 8, 2024, 86 patients (median age 64 years [55-71]; 37 [43%] female and 49 [57%] male) were enrolled and treated in the cycle 1 optimisation cohort. The incidence of cytokine release syndrome was 49% (42 of 86 patients; eight [9%] grade 2; none of grade 3 or worse), with no reported immune effector cell-associated neurotoxicity syndrome.
INTERPRETATION
Epcoritamab monotherapy showed clinically meaningful activity in patients with multiply relapsed or refractory follicular lymphoma, and had a manageable safety profile.
FUNDING
Genmab and AbbVie.
PubMed: 38889737
DOI: 10.1016/S2352-3026(24)00166-2 -
European Heart Journal. Case Reports Apr 2024Atrial fibrillation is a common cardiac arrhythmia and often develops secondary to structural cardiac changes. Both the occurrence of atrial fibrillation and/or...
BACKGROUND
Atrial fibrillation is a common cardiac arrhythmia and often develops secondary to structural cardiac changes. Both the occurrence of atrial fibrillation and/or structural changes of the heart may lead to development of atrial cardiomyopathy and heart failure (HF). However, isolated atrial cardiomyopathy caused by focal atrial thickening is a rare condition, previously only described in case reports as a result of different aetiologies all linked to inflammation.
CASE SUMMARY
A patient with inflammatory-mediated atrial cardiomyopathy causing atrial fibrillation and acute decompensated HF presented as isolated left atrial wall thickening on transoesophageal echocardiography. The diagnosis was confirmed using multimodality imaging with transthoracic and transoesophageal echocardiography, cardiac magnetic resonance imaging, positron emissions tomography/computer tomography scanning and intracardiac echocardiography-guided endomyocardial biopsy. Despite no specific histological aetiology, the observed atrial cardiomyopathy might be associated with type 1 diabetes mellitus. The patient in the present case was successfully treated with prednisolone.
DISCUSSION
Diabetes mellitus is an important risk factor for developing atrial fibrillation and diabetic cardiomyopathy, due to reduced levels of anti-inflammatory and increased levels of proinflammatory cytokines causing cardiac inflammatory structural remodelling. The regression of the atrial thickening might be due to prednisolone's anti-inflammatory effects and thereby ability to suppress atrial remodelling and reduce the occurrence of atrial fibrillation. However, the effect of prednisolone might only affect the non-manifested inflammatory-mediated atrial remodelling. Due to the rare occurrence of isolated atrial cardiomyopathy a multiple imaging approach during the diagnostic process and follow-ups are essential to determine the aetiology and effect of the treatment.
PubMed: 38887777
DOI: 10.1093/ehjcr/ytae167 -
Clinical Case Reports Jun 2024The immunomodulatory effect of CMV makes coinfection with other microbes, like VZV possible and potentially deadlier in the post kidney transplant period. Treatment...
KEY CLINICAL MESSAGE
The immunomodulatory effect of CMV makes coinfection with other microbes, like VZV possible and potentially deadlier in the post kidney transplant period. Treatment should be started promptly. Both infections can be treated with Valganciclovir.
ABSTRACT
Infections are common complications in kidney transplant recipients owing to the lifelong immunosuppression. Cytomegalovirus (CMV) and Varicella Zoster Virus (VZV) infections are quite common in the posttransplant period. Coinfection with both however has been reported only once. The immunomodulatory effect of CMV makes their interaction with other organisms like VZV potentially sinister. This is a case of a young woman who developed coinfection with HZV and CMV in the first month following a live related kidney transplantation from her mother. Transplant surgery went well with good urine output, but serum creatinine did not fall below 1.7 mg/dL. Immunosuppression consisted of intravenous (IV), followed by oral prednisolone, Mycophenolate Sodium (MPS) and Tacrolimus. 25 days after an uneventful surgery, she developed fever, followed by pain and vesicular eruption on the forehead, typical of VZV infection, along with rising creatinine. CMV PCR yielded 300 copies/mL of DNA, which was undetectable in both donor and recipient pre-transplant. Total white blood cell count fell to 2 × 10/L. MPS was temporarily stopped. Treatment with Valgancyclovir led to resolution of fever, skin lesions and brought serum creatinine down to baseline over 2 weeks.
PubMed: 38887304
DOI: 10.1002/ccr3.9089 -
BMJ Case Reports Jun 2024Rheumatoid pleurisy is common in patients with rheumatoid arthritis, but distinguishing it from other diseases, such as heart failure and tuberculous pleurisy, is often...
Rheumatoid pleurisy is common in patients with rheumatoid arthritis, but distinguishing it from other diseases, such as heart failure and tuberculous pleurisy, is often difficult. A man in his 70s with stable rheumatoid arthritis presented with cardiac enlargement and bilateral pleural effusion on chest radiography. Pleural fluid studies showed lymphocytosis, adenosine deaminase level of 51.6 U/L and rheumatoid factor level of 2245.3 IU/mL, suggestive of rheumatoid pleurisy and tuberculous pleurisy. Thoracoscopy under local anaesthesia revealed erythema of the parietal pleura, small papillary projections and fibrin deposits. H&E-stained biopsy specimens showed inflammatory granulomas with strong lymphocytic infiltration and non-caseating granulomas. He was diagnosed with rheumatoid pleurisy. His symptoms improved with 30 mg of prednisolone. This study highlights that biopsy using thoracoscopy under local anaesthesia effectively diagnoses rheumatoid pleurisy, which may be challenging to diagnose.
Topics: Humans; Male; Thoracoscopy; Anesthesia, Local; Pleurisy; Aged; Biopsy; Thoracic Wall; Diagnosis, Differential; Arthritis, Rheumatoid; Prednisolone; Pleura
PubMed: 38885997
DOI: 10.1136/bcr-2024-260140 -
New Zealand Veterinary Journal Jun 2024An 8-month-old male, entire, mixed-breed dog was presented with a 1-month history of left exophthalmos and green mucopurulent ocular discharge. Subsequently,...
CASE HISTORY
An 8-month-old male, entire, mixed-breed dog was presented with a 1-month history of left exophthalmos and green mucopurulent ocular discharge. Subsequently, exophthalmos resolved but esotropia (medial strabismus) developed in the left eye, prompting referral to an ophthalmologist.
CLINICAL FINDINGS
At the initial referral consultation, enophthalmos and esotropia of the left eye were identified. The patient showed mild improvement after a 3-week tapering course of oral prednisolone and doxycycline. MRI was performed and showed left medial rectus muscle atrophy with increased contrast enhancement which was consistent with chronic extraocular muscle myositis (EOM). A forced duction test was performed to confirm the diagnosis of fibrosing esotropia, which is likely a sequela of chronic EOM.
DIAGNOSIS
Fibrosing esotropia presumably caused by untreated EOM.
TREATMENT AND OUTCOME
One month later, esotropia progressed to a marked ventro-medial strabismus resulting in visual deprivation. Surgical release of the ventral oblique, medial and ventral recti muscles was performed, resulting in immediate resolution of the enophthalmos. Despite a tapering post-operative course of oral prednisolone, mild esotropia was present 4 weeks later. In an effort to stabilise the globe position, the low dose of prednisolone was increased to a higher anti-inflammatory dose before slowly tapering over 2 months. The vision in the left eye was improved after surgery and has been maintained since without further treatment.
CLINICAL RELEVANCE
This is the first documented case of fibrosing esotropia in a young dog with prior signs of acute exophthalmos. Fibrosing esotropia has been documented in certain breeds or as a sequela to chronic EOM. In this patient, it was presumably caused by EOM, which was strongly supported by the case history, progression and MRI findings. Most historical reports of EOM described it as a bilateral condition that resolves with systemic corticosteroids at an anti-inflammatory dose. EOM has been shown to also present unilaterally and it can progress to strabismus if not promptly recognised and treated with systemic steroids. Surgical management can restore vision when severe strabismus results in visual deprivation.
PubMed: 38885962
DOI: 10.1080/00480169.2024.2361619 -
CEN Case Reports Jun 2024Red yeast rice has been used to produce alcoholic beverages and various fermented foods especially in East Asia. Since around March 2024, there have been many cases of...
Red yeast rice has been used to produce alcoholic beverages and various fermented foods especially in East Asia. Since around March 2024, there have been many cases of kidney dysfunction in people who have taken certain supplements containing red yeast rice in Japan. We experienced a case of acute kidney injuries induced after taking a supplement containing red yeast rice. A 58-year-old woman was admitted to our hospital due to renal dysfunction suspected to be caused by taking the supplement Benikoji CholesteHelp, which contains red yeast rice. With elevations of urinary tubular injury markers such as urinary β2-microglobulin and N-acetyl-β-D-glucosaminidase, serum creatinine levels were elevated up to 2.75 mg/dL. A kidney biopsy revealed a diagnosis of tubulointerstitial nephritis with lymphocytic infiltration of the interstitium, tubular atrophy, and interstitial fibrotic changes. After discontinuation of the supplement and initiation of the prednisolone treatment, renal dysfunction rapidly improved. The course of this case suggests tubular damage caused by the supplements containing red yeast rice. For early diagnosis and treatment, it should be noted that even what are regarded as nutritional health supplements can cause renal dysfunction.
PubMed: 38884896
DOI: 10.1007/s13730-024-00897-9 -
Clinical Case Reports Jun 2024The co-occurrence of myasthenia gravis (MG) and lichen planus (LP) is a rare phenomenon, with only 13 cases reported in the English literature between 1971 and 2024....
KEY CLINICAL MESSAGE
The co-occurrence of myasthenia gravis (MG) and lichen planus (LP) is a rare phenomenon, with only 13 cases reported in the English literature between 1971 and 2024. Patients with MG or LP, regardless of the thymoma status, require close monitoring for other autoimmune diseases.
ABSTRACT
Myasthenia gravis (MG) is an uncommon autoimmune disease, resulting in fatigable muscle weakness in the ocular, bulbar, and respiratory muscles, as well as muscles of the extremities. Lichen planus (LP) is an autoimmune mucocutaneous disease, presenting with pruritic and violaceous plaques on the skin and mucosal surfaces. So far, MG and LP co-occurrence is only reported in anecdotal individuals. This study reports a patient with MG and LP and systematically reviews the English literature on this rare co-occurrence from 1971 to 2024, indicating only 13 cases with similar conditions. A 67-year-old man presented with ocular and progressive bulbar symptoms, a year after being diagnosed with generalized LP. Laboratory evaluations were normal except for the high anti-AchR-Ab titer and a positive ANA titer. Neurologic examinations revealed asymmetric bilateral ptosis, weakness and fatigability in proximal muscles, and a severe reduction in the gag reflex. He was diagnosed with late-onset, seropositive MG. The treatment included pyridostigmine (60 mg, three times daily), intravenous immunoglobulin (25 g daily for 5 days), and oral prednisolone. There was no evidence of thymoma in the chest x-ray and CT scan without contrast. However, a CT scan with contrast was not performed due to the patient's unstable condition. A common autoimmune mechanism may underlie the unclear pathophysiology of MG and LP co-occurrence, with or without thymoma. Patients with MG, LP, or thymoma require close monitoring and assessment for other possible autoimmune diseases.
PubMed: 38883218
DOI: 10.1002/ccr3.9065 -
Cureus May 2024Erythroderma, also known as exfoliative dermatitis, is a rarely reported atypical cutaneous manifestation of adult-onset Still's disease (AOSD). We present the case of...
Erythroderma, also known as exfoliative dermatitis, is a rarely reported atypical cutaneous manifestation of adult-onset Still's disease (AOSD). We present the case of erythroderma in association with AOSD that was steroid dependent and responded to tocilizumab therapy. Skin rash, pruritis, and related laboratory findings were significantly improved upon the addition of tocilizumab, while prednisolone was successfully tapered to an ever-lowest maintenance level. To our knowledge, this is the first to report the sole therapeutic effect of tocilizumab in erythroderma related to AOSD.
PubMed: 38883073
DOI: 10.7759/cureus.60372 -
Cureus May 2024Isolated herpes zoster optic neuritis is a rare sequelae of herpes zoster ophthalmicus (HZO). It can occur in the acute phase of HZO, or as post-herpetic complications....
Isolated herpes zoster optic neuritis is a rare sequelae of herpes zoster ophthalmicus (HZO). It can occur in the acute phase of HZO, or as post-herpetic complications. We report a case of a young patient with poorly controlled diabetes who developed herpes zoster optic neuritis one month after the initial skin manifestation despite completing a two-week course of oral acyclovir 800 mg five times a day. He complained of a five-day history of sudden onset, painless left eye blurring of vision. His vision over the left eye was no light perception with the presence of a left relative afferent pupillary defect. Fundus examination of the left eye revealed a swollen optic disc. Magnetic resonance imaging showed minimal fat streakiness over the left orbit. He was treated with one week of intravenous methylprednisolone 1 g/day, followed by a tapering dose of oral prednisolone (1 mg/kg/day) together with oral acyclovir 800 mg five times a day for another week. His visual acuity remained poor with a slight improvement in vision to hand motion.
PubMed: 38883008
DOI: 10.7759/cureus.60387