Introduction We present a case of a gentleman with atypical headache symptoms clinically diagnosed as giant cell arteritis (GCA) and initiated on high dose oral steroids. He subsequently developed progressive neurological deficit including bilateral internuclear ophthalmoplegia (INO), as well as third cranial nerve involvement despite above treatment. He received IV methylprednisolone and demonstrated clinical response, temporal artery biopsy confirmed histological evidence of GCA. The nature of his presentation was atypical of cranial giant cell arteritis. Few reported cases describe INO in the context of GCA, with bilateral manifestation being rarer, especially with additional third cranial nerve involvement. Case description A 66-year-old gentleman presented with a 7-day history of bilateral temporal headaches. He noted prominence of both temporal arteries with mild tenderness during this period. He denied any visual changes, PMR symptoms, and jaw claudication or weight loss. He had been experiencing generalised fatigue and myalgia for the preceding 4 months. He was noted to have raised inflammatory markers (CRP 194, ESR 74) and due to the non-specific nature of headache, concern was for possible meningitis. CT head scan was unremarkable and lumbar puncture and CT-CAP did not demonstrate any abnormality including evidence of infection. He was assessed by the rheumatology team and a clinical diagnosis of GCA was made. He was initiated on prednisolone 60mg daily, and described clinical improvement of headaches over the subsequent day. Inflammatory markers also initially responded to treatment. After 2 days of oral therapy, he developed double vision as well as intermittent headache. CRP remained static at 40. He was seen with the neurology team and was found to have divergent gaze of the left eye with ptosis. Eye movements demonstrated bilateral internuclear opthalmoplegia with involvement of the left third cranial nerve. MRI head scan demonstrated small vessel ischaemic changes but no obvious focal pathology. Due to new visual involvement despite prednisolone 60mg, IV methylprednisolone was administered for a period of 3 days. Ophthalmological symptoms did not progress and CRP reduced to 8. Temporal artery biopsy reported findings consistent with GCA. He was re-established on prednisolone 60mg however intermittent headache recurred and CRP gradually increased. Decision was made to increase prednisolone to 80mg (1mg/kg). At this dose headache resolved and CRP decreased to normal range. Methotrexate was introduced at 20mg weekly in order to facilitate with prednisolone weaning. Within 3 weeks of initial IV methylprednisolone administration, ophthalmological symptoms slowly improved to complete resolution. Discussion Typical manifestion of cranial GCA consists of unilateral temporal headache. Patients can however exhibit other symptoms including bilateral involvement and features of systemic inflammation which may be non-specific. Cases of neurological involvement have also been described, with diplopia and cranial nerve involvement being widely reported. Few case reports have described bilateral internuclear ophthalmoplegia which is syndrome involving the medial longitudinal fasciculus of the brainstem. This is usually associated with multiple sclerosis however any pathology of this anatomical region can result in this clinical picture. This gentleman additionally demonstrated features of third nerve palsy of the left eye which would be in keeping with a more typical cranial nerve involvement in GCA. The hypothesis for his clinical picture would be of reversible localised ischaemia to the brain stem secondary to active inflammation of the supplying vessel. MRI imaging did not identify any focal pathology and as mentioned previously, following treatment, his clinical findings fully resolved in a gradual manner over a period of weeks. In regards to the management of his case, following exclusion of infection and in the absence of visual findings on presentation, he was started on prednisolone 60mg daily (40-60mg dose suggested within current BSR guidelines). He described improvement of his headache and CRP was seen to improve initially. Despite this treatment, he developed features of INO and third nerve palsy as described. Implementation of IV methylprednisolone therapy prevented further progression of his symptoms and subsequent resolution of raised CRP. However restarting prednisolone at 60mg after this appeared to demonstrate incomplete control of his condition, thus it was increased to 80mg. Decision to introduce methotrexate at an early stage was made in anticipation of likely difficulties in weaning prednisolone. He remains under close follow-up to monitor prednisolone weaning. He currently has not had any recurrence of his symptoms. Key learning points GCA can present in an atypical manner and should remain a differential in cases of unexplained headache with associated inflammation. A combination of INO and third nerve palsy is an atypical manifestation of this condition. Due to the nature of his presentation, our gentleman was seen by various specialties including the acute-medical team, rheumatologists and neurologists. Fortunately, a probable diagnosis of GCA was made early and appropriate treatment was initiated. However as this case demonstrates, response to treatment can vary and such adjustments were made to accommodate for this, potentially preventing long term disability. Conflicts of interest The authors have declared no conflicts of interest.
Background:Acutely unwell adult patients with hyperinflammatory hyperferritinaemic states are typically challenging to diagnose. Case series suggest that cardiac involvement may be common (up to 20%) but the phenotype has not been well characterised1.The elevation of cardiac biomarkers suggests cardiac involvement, but are non-specific in acute illness. Cardiac MRI (CMR) offers the ability to characterise the myocardium and identify inflammation, and modern motion-corrected sequences now allow the assessment of patients who may struggle to breath-hold in the recovery from acute illness.Objectives:We report 3 patients who underwent CMR in the acute phase of illness with raised cardiac biomarkers.Methods:Case records of acutely ill patients with hyperferritinaemia from two major London centres were reviewed and cases who had undergone CMR in the acute phase of illness were identified.Results:3 cases were identified from a cohort of 22, we report CMR findings from differing aetiologies of hyperferritinaemic states:Case 1: A female in her 60s presented acutely unwell with fever, swollen joints and salmon pink rash. Ferritin was raised at 50574ug/L (20-300ug/L), troponin I 384ng/L (<34ng/L) and Brain Natriuretic Peptide (BNP) 324ng/L (<159ng/L). Echocardiography was normal. However CMR with T2 mapping revealed several small areas of raised signal consistent with myocardial inflammation. A diagnosis of systemic Adult Onset Stills Disease (AOSD) was made. She received IV methylprednisolone and anakinra with normalisation of cardiac biomarkers.Case 2: A male in his 20s with known SLE with associated end stage renal failure requiring transplant. He had a previous prolonged admission secondary to HLH. He presented with chest pain and concave shaped ST elevation on ECG. Troponin peak 2168ng/L, BNP 1334ng/L. Peak ferritin 1300ug/l.He was initiated on colchicine for likely pericarditis. Echocardiography showed a dilated left ventricle and mildly increased wall thickness, but overall systolic function within normal limits.CMR reported nodular patchy late gadolinium enhancement in the mid inferoseptum and inferior wall associated with areas of raised T2 mapping values. NM cardiac rest gated PET reported abnormal FDG uptake to the myocardium with sites including the apical inferior wall, apical RV insertion point and basal septal/anterior right ventricular walls. Features were deemed in keeping with active myocarditis.He responded to colchicine with improved troponin, and was discharged with close follow up.Case 3: A male in his 20s presented with septic shock attributed to meningococcal septicaemia requiring ITU admission. Troponin was elevated at >9000ng/L. Bloods demonstrated raised ferritin and features consistent with HLH were identified.CMR reported elevated native myocardial T1/T2 signal of the lateral and mid-anterior walls in keeping with myocardial oedema. Pericardium adjacent to the anterolateral wall had elevated T1/T2 signal with hyperenhancement on delayed enhancement imaging. Tissue characterisation was in keeping with an acute myopericarditis process.In addition to broad spectrum antibiotics to treat his underlying infection, he received therapy for HLH including methylprednisolone, anakinra and IVIG. He subsequently made a good recovery to treatment.Conclusion:CMR in acute illness with hyperferritinaemia reveals abnormal tissue characterisation with myocardial inflammation, even when echocardiography is normal. We suggest CMR may be a useful test to expand our understanding of hyperferritinaemic disease states.References:[1]M Gerfaud-Valentin et al. Myocarditis in Adult-Onset Still Disease. Medicine (Baltimore) 2014 Oct; 93(17): 280-289Disclosure of Interests:None declared
Introduction Vasculitis can be a primary or secondary process. Underlying secondary causes include infection, malignancy and other autoimmune conditions. This case describes a patient with findings consistent with ANCA-associated vasculitis. As part of investigations, she was identified as having a PET-avid lung lesion for which she is currently undergoing further investigation via the respiratory team. From a rheumatological perspective she is receiving treatment for her vasculitis, however at present it is unclear whether this is a secondary paraneoplastic phenomenon. This case explores the nature of her presentation and the relationship of primary versus secondary paraneoplastic vasculitis. Case description A 75-year-old lady presented to hospital with a 6-week history of fatigue, pyrexia and myalgia. During this period she additionally noted numbness/neuropathic pains affecting her feet. She had a background of COPD/asthma diagnosed 10 years previously. She had additionally undergone polypectomy for nasal-polyps and had underwent right mastectomy in 1990 for breast cancer. She was noted to have raised inflammatory markers (CRP 256) and eosinophilia. She was deemed to have an infective exacerbation of COPD/asthma although at the time she had limited respiratory symptoms and CXR was reported normal. She was initiated on a 5-day course of prednisolone 40mg and antibiotics. Her symptoms appeared to respond to treatment, as did her CRP and eosinophilia, and she was discharged. Unfortunately she re-presented with similar symptoms within a few days. Further investigation and blood tests demonstrated cANCA antibodies with PR3 of 128 IU. Urine PCR was mildly raised at 30mg/mmol. She was reviewed by the rheumatology team, and likely diagnosis of ANCA-associated vasculitis was made. It was noted however that while her serology was suggestive of granulomatosis with polyangiitis (GPA), her clinical picture of eosinophilia, COPD/asthma and nasal-polyps was more consistent with eosinophilic granulomatosis with polyangiitis (EGPA). She was re-initiated on prednisolone 40mg and demonstrated clinical response. As part of work-up, CT-CAP was arranged. This reported a spiculated lesion of the right-upper lung lobe with appearances concerning for malignancy. Subsequent PET-CT confirmed this be PET-avid, with additional suspicious nodules. She was additionally arranged for nerve-conduction studies regarding her feet symptoms, with findings being consistent with mononeuritis multiplex. From a rheumatological perspective, she has been initiated on azathioprine and prednisolone is gradually being reduced. She has ongoing follow up with respiratory regarding her lung lesion, with initial biopsy being inconclusive. Further attempt at gaining histology is currently awaited. Discussion From a rheumatological perspective, aspects of this case support a diagnosis of ANCA-associated vasculitis. Presence of cANCA with PR3 antibodies are typically associated with GPA, although her eosinophilia, asthma and previous history of nasal polyps would be more consistent with EGPA. The features of mononeuritis multiplex on nerve conduction studies would also support an underlying diagnosis of vasculitis. Although there is a documented relationship between malignancy and vasculitis, this appears to be relatively rare with a 5-8% association. Haematological malignancies such as lymphoma and MDS have a greater association in comparison to solid tumours. Typical manifestation of paraneoplastic vasculitis is that of cutaneous involvement, particularly of leukocytoclastic vasculitis. Reports suggest improvement of secondary vasculitis on treatment of the underlying condition. With regards to the association of ANCA-vasculitis and solid tumour malignancy, cases appear to be less well-described. The majority of case studies exploring the link between these conditions are primarily in the context of secondary malignancy occurring after immunosuppressive therapy (such as cyclophosphamide) for vasculitis. Review of literature outside of this context, especially in regards of specifically lung malignancy is limited – so a consistent established link may be less apparent. Of the limited studies, interestingly there have been reports of patients with biopsy proven lung malignancy and raised MPO/PR3, with levels normalising following treatment of the underlying lesion. As described in this case, our patient currently awaits further investigation regarding her lung lesion and subsequent review of its definitive intervention. Should investigations confirm malignancy and this be treated, then it would be interesting to see whether there is clinical resolution of her systemic vasculitis secondary to this. Key learning points This case demonstrates the presence of 2 conditions of ANCA-associated vasculitis and possible lung malignancy which were both diagnosed during the same admission. Literature suggests possible association between vasculitis and malignancy in a general sense, however specific description of ANCA-vasculitis and lung malignancy has been rarely described. At present, this patient’s vasculitis is being managed whilst definitive intervention is being arranged for her underlying lung lesion. Continued follow up from both the rheumatologists and respiratory team is ongoing and response of her condition will be assessed pending intervention of her underlying lung lesion. Conflicts of interest The authors have declared no conflicts of interest.
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