Dose Intravenous Methylprednisolone Research Articles

A Rare Case of Pembrolizumab Associated Encephalopathy

We present a unique case of a 55-year-old man with confusion thought to be due to pembrolizumab which he was receiving for renal cell carcinoma. His workup for other possible etiologies for encephalopathy was negative. He was treated with high dose intravenous methylprednisolone followed by prednisone taper and intravenous immunoglobulin with gradual improvement in his mentation.

Multisystem inflammatory syndrome in children in Coastal Andhra, India

Background: Multisystem inflammatory syndrome in children (MIS-C) associated with COVID is a recently emerged disease and there is limited Indian data. We reported the presentation and clinical course of these children.Methods: All children who had symptoms and signs of MIS-C according to WHO criteria and those positive for SARS-CoV-2, were considered for the study. Clinical features, lab parameters, treatment details and outcome were documented.Results: 104 children, who had MIS-C during the study period. Out of them, 52 (50%) required respiratory support. Among those who required respiratory support 13 (12%) children required invasive ventilation, 11 (10.5%) children could be managed with noninvasive ventilation, whereas 28 (27%) children required supplemental oxygen. 18 (17%) children had signs of myocarditis and left ventricular dysfunction. 12 (11.5%) children had pulmonary edema and Coronary artery dilatation was present in 4(4%) cases. Steroids were used as main stay of therapy. Low dose intravenous methylprednisolone was administered to72(69%); pulse dose methylprednisolone was administered to 12 (12%) children. Intravenous immunoglobulins (IVIG) administered to 20 (19%) children. Among the study group, 103 children survived and one child expired (0.96%).Conclusions: Optimal use of immunomodulators and frequent hemodynamic assessment with functional ECHO to optimize the inotropes, can improve the outcomes among those with myocardial dysfunction.

COVID-19 Infection or Buttock Injections? The Dangers of Aesthetics and Socializing During a Pandemic

IntroductionSilicone (polydimethylsiloxane) injections are used for cosmetic augmentation. Their use is associated with life-threatening complications such as acute pneumonitis, alveolar hemorrhage, and acute respiratory distress among others [1,2]. We report a case of a Hispanic woman who developed severe respiratory distress syndrome after gluteal silicone injections.Case PresentationA 44-year-old Hispanic female presented to the Emergency Department complaining of progressive dyspnea on exertion for two weeks. Chest imaging revealed patchy bibasilar airspace opacities of peripheral distribution. Labs were significant for leukocytosis, elevated PT, D-dimer, lactate dehydrogenase, and fibrinogen, concerning for COVID-19, however SARS-CoV-2 testing was negative multiple times. The patient later became encephalopathic, hypoxemic, and eventually required intubation. Further history uncovered that the patient had received illicit gluteal silicone injections a few days prior to her onset of symptoms. The patient was diagnosed with silicone embolism syndrome (SES) and initiated on high dose intravenous methylprednisolone [1].Case DiscussionPatients from lower socioeconomic backgrounds utilize illicit services to receive silicone injections at minimal costs. This leads to dangerous outcomes. The serology and imaging findings observed in our case have similarities to the typical presentation of COVID-19 pneumonia making the initial diagnosis difficult. This case serves as a cautionary tale of the importance of thorough history taking in patients with concern for COVID-19.

A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes

Ocular involvement in cases of bullous pemphigoid is rare and when present, the signs are usually subtle and in the form of fine tarsal scarring and dry eye disease. The current report aims to describe the clinical features and management protocols in a series of cases with aggressive ocular manifestations at presentation. All cases of bullous pemphigoid seen between 2017 and 2020 were included in this retrospective case series. Data regarding the clinical features, treatment administered, and outcomes was collected. Five cases (n=10 eyes) of bullous pemphigoid disease with ocular involvement were included. All eyes had significant cicatricial conjunctival changes in the form of symblephara, inferior forniceal shortening, and tarsal conjunctival scarring. Conjunctival granulomas were present in 3/10 eyes. Corneal involvement in the form of punctate keratitis was present in all eyes while 4/10 eyes had an epithelial defect as well. The management of these cases involved topical therapy with corticosteroids and lubricants (n=10 eyes) while pulse doses of intravenous methyl prednisolone were administered in 5/5 cases. Pulse intravenous cyclophosphamide was supplemented in 2/5 cases. Adequate control of the disease was noted in 3/5 cases while one case had a recalcitrant form of the disease and developed a dermalised ocular surface in both eyes. The last patient was lost to follow up during the course of therapy. Bullous pemphigoid can present with an aggressive form of cicatrizing conjunctivitis similar to other variants of autoimmune blistering disorders and must be considered as a differential in cases presenting with ocular cicatricial disease. Long-term intensive immunosuppression is required for the management of these cases to preserve the visual function and the integrity of the globe.

Stroke-Like Migraine after Radiation Treatment Syndrome in Children with Cancer

AbstractStroke-like migraine attacks after radiation therapy (SMART) syndrome is a symptom complex of transient neurological deficits, headache, and abnormal cortical contrast enhancement on brain MRI. Pathophysiology is unclear, but exposure to cranial radiation (RT) is a sine qua non. We report five children with SMART syndrome treated with RT therapy for medulloblastoma (n = 3), atypical teratoid rhabdoid tumor (n = 1), and pleomorphic xanthoastrocytoma (n = 1). Median age at tumor diagnosis was 9.4 years (range 5.1–14.7). Median follow-up from cancer diagnosis was 3.1 years (range 1.4–12.9). All patients had 54 Gy focal RT treatment and medulloblastoma children had additional 36 Gy craniospinal irradiation. Median time from the end of RT to first transient neurological deficit was 1 year (range 0.7–12.1). The median follow-up since first SMART episode was 0.6 years (range 0.3–2.6). Presenting symptoms included the gradual development of unilateral weakness (n = 4), non-fluent dysphasia (n = 1), somnolence (n = 1), and headaches (n = 3). Neurological deficits resolved within 30 minutes to 10 days. Transient cortical enhancement on magnetic resonance imaging (MRI) was confirmed in two children and was absent in the other three. Two children had a single and three had multiple episodes over the next few months. Two children with protracted symptoms responded to 3 days treatment with high dose intravenous methylprednisolone. Symptoms ultimately resolved in all patients. SMART syndrome is a rare disorder characterized by slow evolution of neurological deficits with variable abnormal cortical contrast enhancement. The use of steroids may improve symptoms and speed resolution.

Role of high dose intravenous methylprednisolone and visual evoked potential (VEP) in the management of methyl alcohol poisoning: A case series report

To study the effectiveness of high dose intravenous methylprednisolone and to discuss the role of visual evoked potential (VEP) as a marker of severity and of improvement in methyl alcohol poisoning.In an outbreak of methanol poisoning, a total of six patients presented to JNMCH, AMU. Meticulous history was taken followed by examination. They were treated with high dose IV methyl prednisolone and later underwent visual evoked potential testing. The patients were examined daily till the time of discharge and then followed up at 1 month.Following treatment with high dose IV methyl prednisolone, 100% of cases showed improvement in vision which was judged on the basis of visual acuity and visual evoked potential assessment at the time of presentation. As recorded by VEP, 4 patients (66.6%) had prolonged P100 latency while no response was recorded in 2 patients (33.3%). In 3 (50%) of the 6 cases, improvement in visual acuity of at least two lines of Snellen chart was observed at 1 month follow-up while two patients (33.3%) improved from doubtful light perception to counting fingers at least 2 metres and one patient (16.6%) improved from perception of light present to counting finger at least 2 metres.Early presentation and timely intervention improves visual outcome in methanol poisoning. High-dose IV methylprednisolone seems to have benefits in the treatment of methanol optic neuropathy. The VEP examination appears to be a sensitive marker for diagnosis and prognosis of even subclinical impairment of the optic system due to methanol poisoning.

Anti Zic4 Paraneoplastic Neurological Syndrome Presenting as Oscillopsia.

Anti Zic4 Paraneoplastic Neurological Syndrome Presenting as Oscillopsia.

MO225CMV ENCEPHALITIS IN A SLE FLARE

Abstract Background and Aims Cytomegalovirus (CMV) infection is an opportunistic pathogen in immunocompromised patients and its management is well described after solid organ/bone marrow transplant or in HIV patients. Although less common, since Systemic Lupus Erythemathous (SLE) is a chronic auto immune disease often requiring intense immunosuppression to induce remission of disease exacerbation, SLE patients are also prone to it. Besides complicating the course of the disease, it may be a life-threatening infection. Due to the heterogeneity of SLE manifestations, usually is difficult to distinguish between a SLE flare and CMV active infection, making the diagnose challenging. It has also been described in the literature as an exacerbating SLE factor. We report the case of an Asian, 27-year-old woman, with a recent SLE diagnosis that was admitted with a SLE flare while developing a CMV encephalitis. She was previously admitted due to a nephrotic syndrome. Immunological studies revealed an ANA title of 1:640 and a positive anti dsDNA Ab with diminished C3 and C4 levels, nephrotic range proteinuria and a diagnose of SLE was made. Renal biopsy revealed class IV lupus nephritis. She started high dose intravenous methylprednisolone (3 pulses of 500mg) and mycophenolate mofetil (MMF) at a dose of 2g per day. She was discharged taking 60 mg of oral prednisolone and the same dose of MMF. Two weeks after being discharged, she was readmitted due to worsening anaemia (Hb 6.8 g/dL), thrombocytopenia (Pl 27 000/µL) and deteriorating renal function with a sCr of 5.5 mg/dL with de novo haematuria. It was admitted a severe SLE flare and she was given another 3 pulses of 500mg I.V methylprednisolone and cyclophosphamide (CYC) was started (1 pulse of 500mg I.V). At the same time, she started to complain of myalgias and malaise, generalized hypotonia, developed fever, leukopenia with neutropenia and seizures. Serum CMV viremia was 71 000 copies and CMV polymerase chain reaction was positive in cerebrospinal fluid. She was started on I.V Ganciclovir, CYC was suspended and clinical improvement was observed. Conclusion Studies about the risk of different treatment drugs and other risk factors on the development of CMV disease in SLE are lacking. These studies will be useful for establishing guidelines on the institution of prophylaxis or pre-emptive treatment of CMV infection in SLE patients. Protocols for screening and prevention in this population should be implemented to account for this emerging problem. Given the rising prevalence of CMV infection in the past few years, the authors recommend that patients recently diagnosed with SLE while taking high doses of corticosteroids, which appears to be a risk factor for CMV reactivation, should be routinely tested for CMV viremia. There should be a low threshold for suspicion, hence treatment should be started as soon as possible given the high morbidity and mortality in severe cases.

P047 An unusual case of dermatomyositis with co-existent anti-GAD and anti-NXP2 antibody positivity

Abstract Background/Aims Dermatomyositis (DM) is a rare disease. We present a case of DM with both anti-nuclear matrix protein 2 antibody (NXP2) as well as anti-glutamic acid decarboxylase antibody (GAD) positivity, the combination of which has yet not been documented in the current literature. Methods The case is described below. Results A 48 year old Caucasian male with no co-morbidities presented with a one-month history of sore throat and lethargy, followed 2 weeks later by muscular pain in his upper arms. On examination, there was erythema to the sun-exposed areas of the face and arms, hoarse voice and Gottron’s papules. He had proximal muscle weakness of 4/5 in his shoulder and hips. His creatinine kinase(CK) was elevated at 2914U/L. He was started on methylprednisolone 1 gram od for 3 days with improvement of his CK to 642U/L. His muscle biopsy showed mild chronic neurogenic changes and upregulation of C5b-9 but no evidence of inflammatory myopathy which was attributed to the steroid use prior to the biopsy. His myositis antibody screen was positive for Anti-Nuclear Matrix Protein antibody 2(NXP2). After 4 days he developed rapid onset dysphagia and a sudden drop in his spirometry readings with a fall in his FEV1from 94% to 85% and a fall in his FVC from 88% to 79%. He became acutely unwell, drowsy with difficulty completing sentences and global weakness. His CK had risen to 1567U/L despite the high dose intravenous methylprednisolone. He was diagnosed with Diabetic ketoacidosis(DKA) with a blood glucose of 32mmol/L , pH of 7.0, and transferred to ITU. Intravenous immunoglobulin (IVIG) was administered with subsequent improvement in all muscle groups. He was then commenced on a subcutaneous insulin regime and pulsed intravenous cyclophosphamide (Eurolupus) as his EMG showed a sensorimotor polyneuropathy as well as an inflammatory myopathy. His serology was negative for infectious aetiology. Malignancy screen including PET scan, oesophagogastroduodenoscopy and colonoscopy were normal however, he was found to be positive for anti-GAD antibody. Conclusion GAD antibodies are known to be associated with many disorders including diabetes and stiff person syndrome and can recognise different epitopes in various diseases. This is the first documented case of GAD occurring in a DM patient, in the presence of NXP2 antibodies. IVIG is used to treat both stiff man syndrome and refractory DM. It is possible that the combination of GAD and NXP2 contributed to the pathogenesis and severity of the clinical presentation in this case. Disclosure K. Beharry: None. G. Barminski: None. D. De Lord: None.

Rituximab for refractory necrotizing scleritis and peripheral ulcerative keratitis secondary to antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis

A 52-year-old woman was diagnosed with bilateral necrotizing scleritis (NS) and peripheral ulcerative keratitis secondary to the antineutrophil cytoplasmic antibody (ANCA)-negative granulomatosis with polyangiitis (GPA) as evidenced by increased erythrocyte sedimentation rate, bilateral multiple cavitation lung nodules, and persistent microscopic hematuria. Infective workup and autoimmune tests were negative including the ANCA test. She received multiple courses of high dose intravenous methylprednisolone, intravenous cyclophosphamide, and oral methotrexate. Multiple tectonic patching operations were performed. Despite all the treatment, the disease continued to progress. Eventually, few doses of rituximab were given and the disease was stable for at least a year without any reactivation.

“Triple-positive” renal limited vasculitis presenting with rapidly progressive glomerulonephritis

Rationale: Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) in a case of glomerulonephritis is often identified as a “double-positive” disease. Interestingly, the majority of “double positive” ANCA is myeloperoxidase (MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis, suggesting an ANCA-associated kidney lesion. Proteinase 3-ANCA positive diseases are also rarely reported. Patients positive for all three antibodies, i.e., triple-positive patients, are extremely rare. Patient's Concern: A 53 year-old female presented with anasarca and oliguria of 2 months’ duration. Diagnosis: Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA, proteinase 3-ANCA, and anti-GBM antibody (triple-positive). Interventions: Intravenous high dose cyclophosphamide, oral azathioprine, intravenous methylprednisolone, and plasma exchange as per British Health Professionals in Rheumatology Guidelines. Outcomes: After one-month follow-up, anasarca and proteinuria were lessened, serum creatinine was normalized, titers of MPO-ANCA levels were decreased, and anti-GBM antibody levels were normalized. Lessons: Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.

Myelin Oligocytes Glycoprotein (MOG) antibody positive bilateral optic perineuritis in a young male treated with steroids and fingolimod

A 25 year old male, chronic smoker presented with sudden diminution of vision and pain on ocular movement in both eyes. Fundus examination revealed both eyes optic disc swelling. Patient responded well to pulse dose intravenous Methylprednisolone (IVMP). MRI showed both eyes mild optic nerve sheath enhancement. Three month later, he came with blurring of vision in both eyes with right eye lateral rectus palsy. Atypical panel revealed Anti-MOG positive in serum. He was given IVMP and is started on oral Fingolimod, and is stable at present. We report a case of recurrent bilateral optic perineuritis with sero-positive MOG autoantibody and abducens nerve palsy.

Screening for urinary tract colonisation prior to corticosteroid administration in acute multiple sclerosis relapses: Validation of an updated algorithm

IntroductionTo evaluate an updated algorithm in the detection of urinary tract infection (UTI) prior to high-dose corticosteroid treatment in acute relapses in multiple sclerosis (MS). This updated algorithm aimed to decrease the unnecessary use of antibiotics, whilst maintaining accuracy and safety. MethodsProspective cohort study of 471 consecutive patients with MS relapses in a hospital-based outpatient acute relapse clinic. 172 patients met exclusion criteria, leaving 299 patients for analysis. Patients underwent urine dipstick and were treated for UTI if 2 or more of: nitrites, leukocyte esterase and cloudy urine were positive. Patients with confirmed acute MS relapse were treated with high dose intravenous or oral methylprednisolone. ResultsSignificant bacteriuria (>105 colony forming units/mL) was present in 33 (11%, 95% CI 8-15) patients. The algorithm sensitivity and specificity was 24% and 94% respectively; the negative predictive value was 91%. The overall accuracy of the algorithm was 87%. No adverse sequelae were identified in 25 patients who received high dose methylprednisolone in the presence of an untreated UTI. ConclusionWith an improved specificity, this updated algorithm addresses previous issues concerning the unnecessary prescription of antibiotics, whilst improving accuracy and maintaining safety.

27. A new presentation of interstitial lung disease

27. A new presentation of interstitial lung disease

54. Binocular sight loss: uncommon, yet a devastating complication of GCA

IntroductionGCA (giant cell arteritis) affects cranial branches of the arteries from aortic arch, especially the superficial temporal artery and vessels supplying the eye. Ophthalmic manifestations of GCA are characterised by the vasculitis of the ophthalmic artery and its branches. We present a case of a patient who developed the binocular sequential permanent vision loss secondary to GCA due to the delayed diagnosis.Case descriptionA seventy-six year-old lady with a background of type two diabetes mellitus, hypertension and dyslipidemia and hemicolectomy for appendix tumour two years ago (no recurrence) was admitted with blurring of vision in the eye. She did not complain about other symptoms, however, on direct questioning she admitted six weeks history of weight loss (2.5 stones), severe tiredness and occasional night sweats, mild left sided temporal headache. She was initiated on 60mg of prednisolone. One week prior to admission she had right eye CRAO diagnosed by the ophthalmology team with preceding three weeks history of right eye blurring of vision. CRP at that time was 12 g/L and GCA was not considered likely. Following her permanent right sided vision loss, she was referred to stroke team for further management and was given aspirin. Her CT and MRI brain scans were unremarkable, USS carotid arteries revealing diffuse intimal thickening in all the arteries of the neck. On assessment in the rheumatology fast track clinic she denied any associated jaw/tongue/arms claudication and no proceeding or historical PMR symptoms. She had mild left sided temporal artery tenderness and decreased temporal artery pulses bilaterally, no vision in the right eye along with the blurring in the left eye. There were no audible bruits in carotid, axillary, femoral arteries. Her acute phase maker (CRP) was mild but persistently elevated with a maximum level of 12 g/L for one week prior the admission. USS showed features consistent with GCA with extensive non-compressible halo signs in temporal arteries and its branches bilaterally. An urgent ophthalmology review revealed AION changes in the left eye, her vision deteriorated over the next forty-eight hours despite the initiation of high dose intravenous methylprednisolone. The patient was also started on tocilizumab infusion in an attempt to reverse the sight loss.DiscussionThe ophthalmic manifestations of GCA range from AION (anterior ischaemic optic neuropathy), CRAO (anterior central retinal artery occlusion), cilioretinal artery occlusion to occipital lobe infarcts, transient monocular vision loss, photopsias or diplopia. Ophthalmic GCA is an emergency and requires urgent ophthalmological evaluations and initiation of treatment with high dose of steroids to avoid permanent loss of vision. The reported incidence of visual symptoms in GCA ranges widely from 12% to 70% of cases. GCA is often associated with constitutional symptoms such as weight loss, night sweats, fevers, systemic inflammatory response with elevated inflammatory markers. However, it had been reported that this can be very mild in ischaemic GCA.The diagnosis of GCA in this particular case was delayed for approximately six weeks due to the atypical initial presentation with mainly constitutional symptoms and unilateral vision blurring as well as mildly elevated inflammatory markers. A collaboration of three teams (namely ophthalmology, stroke and rheumatology) proved necessary to initiate high dose steroid treatment and tocilizumab. However, patient still suffered from permanent binocular vision loss. She was registered blind and will in future require extensive physical and psychological support.Key learning pointsDespite the recent advances and increased awareness with good availability of fast track GCA services, cases of bilateral blindness are still observed. This is due to a lack of awareness of atypical non-cranial symptoms that frequently accompany a GCA ischaemic presentation. Inflammatory markers are often only mildly elevated in such cases. We suggest a heightened public and professional awareness program to mitigate this irreversible disastrous complication.Conflicts of interestThe authors have declared no conflicts of interest.

Anti-NMDAR encephalitis with simultaneous hypertrophic pachymeningitis in a 68-year-old male: a rare case report

BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most frequent types of autoimmune encephalitis. However, the instigating mechanisms are as yet not fully ascertained. Cardinal clinical manifestations of anti-NMDAR encephalitis include acute behavioural change, psychosis, and catatonia. As the level of diagnosis increases, encephalitis becomes more common, but there are never been published in patients with anti-NMDAR encephalitis and simultaneous hypertrophic pachymeningitis.Case presentationA sixty-eight-year-old man who presented with mental, behavioral abnormalities, unstable walking, headaches, and erratic hand movements. The neuropsychiatric symptoms and cerebrospinal fluid examination was consistent with the diagnosis criteria of anti-NMDAR encephalitis. Magnetic resonance imaging of the brain showed a thickening of dura mater localized at the left tentorium cerebelli, left cerebral hemisphere, and cerebral falx; the thickening dura mater was characterized by an intense contrast enhancement after the administration of gadolinium. High doses of intravenous methylprednisolone were administrated during his hospitalization. After 5 days, the patient’s condition improved.ConclusionsWe herein describe a rare case of a 68-year-old man with anti-NMDAR encephalitis presenting with concomitant hypertrophic pachymeningitis. We systematically expounded anti-NMDAR encephalitis and hypertrophic pachymeningitis, and made bold conjectures on the etiology and pathogenesis of these two diseases, hoping to stimulate new ideas from clinicians and basic medical researchers.

Effect of Treatment on Damage and Hospitalization in Elderly Patients with Microscopic Polyangiitis and Granulomatosis with Polyangiitis.

Age is a risk factor for organ damage, adverse events, and mortality in microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). However, the relationship between treatment and damage, hospitalizations, and causes of death in elderly patients is largely unknown. Consecutive patients from Sweden, the United Kingdom, and the Czech Republic diagnosed between 1997 and 2013 were included. Inclusion criteria were a diagnosis of MPA or GPA and age 75 years or more at diagnosis. Treatment with cyclophosphamide (CYC), rituximab (RTX), and corticosteroids the first 3 months was registered. Outcomes up to 2 years from diagnosis included Vasculitis Damage Index (VDI), hospitalization, and cause of death. Treatment data were available for 167 of 202 patients. At 2 years, 4% had no items of damage. There was a positive association between VDI score at 2 years and Birmingham Vasculitis Activity Score at onset, and a negative association with treatment using CYC or RTX. Intravenous methylprednisolone dose was associated with treatment-related damage. During the first year, 69% of patients were readmitted to hospital. Myeloperoxidase-antineutrophil cytoplasmic antibody positivity and lower creatinine levels decreased the odds of readmission. The most common cause of death was infection, and this was associated with cumulative oral prednisolone dose. Immunosuppressive treatment with CYC or RTX in elderly patients with MPA and GPA was associated with development of less permanent organ damage and was not associated with hospitalization. However, higher doses of corticosteroids during the first 3 months was associated with treatment-related damage and fatal infections.

Testicular ischemia in deficiency of adenosine deaminase 2 (DADA2)

BackgroundDeficiency of adenosine deaminase 2 (DADA2) is a rare autosomal recessive autoinflammatory condition. Recognised features include vasculitis predominantly affecting medium sized vessels, livedoid skin rash, central and peripheral nervous system involvement, variable degrees of immunodeficiency, and marrow failure, amongst other clinical presentations. We present the case of a six year old male with DADA2 who presented with acute testicular ischaemia secondary to vasculitis, the first such description in DADA2.Case presentationA six year old male presented acute right-sided testicular pain. His history included transient infantile neutropenia, resolved hepatosplenomegaly, and longstanding livedo racemosa, leading to screening and confirmation of DADA2 caused by homozygous c.139G > C (p.G47R) mutation of ADA2. As his only clinical feature was that of mild livedo racemosa with normal laboratory parameters at diagnosis, he was being actively monitored prior to starting any treatment. At a routine clinic follow-up a 24 h history of testicular pain was noted on systems review. He was afebrile, and his only physical signs were that of moderate livedo racemosa, and tenderness of the right testicle. Laboratory parameters revealed C-reactive protein (CRP) 8 mg/L (reference range [RR] < 20 mg/L); erythrocyte sedimentation rate (ESR) 28 mm/hr. (RR < 10); and serum amyloid A (SAA)5 mg/L (RR < 10). Ultrasound-scan of the scrotum revealed significantly reduced perfusion of the right testes, without torsion. Surgical scrotal exploration confirmed testicular ischaemia without torsion. Histology demonstrated ischaemic seminiferous tubules with intervening haemorrhage and acute inflammatory cells, consistent with vasculitis of the testis as the cause. He was treated with high dose intravenous methyl-prednisolone followed by a weaning course of oral prednisolone, and subcutaneous adalimumab (anti-tumour necrosis factor alpha, anti-TNFα). Repeat ultrasound-scan 3 weeks later revealed good testicular perfusion, with a small area of focal infarction. At last follow-up (11 months post-event) he remained asymptomatic, on treatment with adalimumab.ConclusionThe phenotype of DADA2 continues to expand, and we add testicular infarction to the features of DADA2. CRP and SAA cannot be relied on as reliable biomarkers to predict tissue ischaemia and hence who to target for anti-TNFα therapy in DADA2, since these remained steadfastly normal before, during, and after testicular infarction in this case.

Substitution of single dose intravenous methylprednisolone (MP) for oral prednisone (Pred) in RCHOP in intermediate grade non-Hodgkin’s lymphoma (NHL).

e19019 Background: The regimen CHOP (cyclophosphamide, Hydroxydaunorubicin [doxorubicin], Oncovin [vincristine] and Pred) has been the basis of therapy given with curative intent for intermediate grade NHL for over 40 years. Rituximab added to CHOP (RCHOP) has been the standard for more than a decade. For patients (pts) with prior medical problems such as brittle diabetes, gastrointestinal bleeding, or psychiatric disorders, oral Pred given daily for 5 days is problematic. Methods: In this retrospective study between January, 2007 and September 2018, twenty pts, Diffuse Large B-cell (18) or grade III Follicular NHL (2), for whom standard oral Pred (100 mg daily for 5 days) was not felt to be medically well-suited or who refused or were not compliant with such therapy following an initial cycle, were treated with curative intent with the substitution of MP 125 mg IV (one received 60 mg IV) on day 1 (in place of oral Pred for 5 days) in combination with standard doses of rituximab 375 mg/m2, cyclophosphamide 750 mg/m2, doxorubicin 50 mg/m2, and vincristine 2 mg (total dose) all given intravenously on day 1 with peg-filgastrim 6 mg subcutaneously on day 2 every 21 days. Results: Pt characteristics: 14 males/6 females, median age-56 (range: 24-76), stages: II (8), III (2), IV (10), B symptoms (12), subtypes: activated B-cell (11), germinal center (6), diffuse B-cell NOS (1), follicular grade III (2). Two were treated with 5 cycles. Fifteen received 6 cycles while three were treated with 8 cycles. Sixteen had complete responses (evaluated by PET/CT) following RCHOP. One pt with activated B-cell subtype had a short-lived partial response before experiencing disease progression. The three remaining pts had partial responses, all 3 of which were converted to complete responses following involved site radiation therapy. One with activated B-cell subtype relapsed at 68.2 months. Eighteen remain progression-free with the longest at 120.3+ months. Median progression-free survival has not been reached at 23+ months. Conclusions: RCHOP with the substitution of a single dose of MP in place of oral Pred has activity in intermediate grade NHL. Long-term disease-free survival may be seen.

Parotid Gland Edema After Chlorhexidine Mouthrinse: Case Report and Literature Review

Introduction: Parotid gland swelling, caused by many pathological conditions, has also been reported to be a possible side effect of the use of chlorhexidine mouthwash. This adverse reaction to chlorhexidine mouthwash is, however, extremely rare and very few cases of parotid gland swelling due to chlorhexidine mouthwash have been reported in the literature. Case Description: This report describes the clinical management of unilateral parotid swelling caused by chlorhexidine mouthwash. Methods: A patient presented with left parotid gland swelling after using chlorhexidine mouthwash for three days following sinus augmentation on the contralateral side of the maxilla. Diagnosis of parotid gland swelling due to rinsing with chlorhexidine was formulated after anamnesis, clinical examination, radiographs and ultrasound of the gland excluded other pathological conditions. The patient was subsequently advised to stop rinsing. However, on the evening of the same day, swelling increased and the patient presented to an emergency department where a single intravenous dose of methylprednisolone was administered. Results: After seven days, parotid swelling decreased significantly and after three weeks had completely disappeared. Conclusion: Although unilateral or bilateral parotid gland swelling related to the use of chlorhexidine mouthwash is an uncommon adverse event, it must be suspected after other organic or infective conditions have been excluded. The precise pathogenic mechanism has not yet been determined and further studies should be carried out to better understand the pathophysiology of this uncommon phenomenon.