A 59-year-old lady with underlying hypothyroidism presented with acute contact dermatitis progressed to cellulitis with superimposed bacterial infection and acute kidney injury. She responded to initial management with antibiotics, but a week later, she had cutaneous and systemic vasculitis. Her skin biopsy consistent with immune-mediated leuko-cytoclastic vasculitis and her blood test was positive for cytoplasmic-anti-neutrophil cytoplasmic antibody (c-ANCA). A diagnosis of ANCA-associated vasculitis was made and she was treated with immunosuppressant with plasmapheresis and hemodialysis support for her kidney failure. Despite aggressive measures, the patient succumbed to her illness. This case report demonstrates that soft tissue infection could trigger the development of ANCA-associated vasculitis whilst a background of hypothyroidism serves as a predisposing factor as both condition were reported separately in a couple of case studies before.
This article aims to report a case of a young female patient with anti-neutrophil cytoplasmic antibodies-associated vasculitis complicated with pulmonary renal syndrome, multiple relapses, and who later developed venous thromboembolism. Pulmonary renal syndrome is a well- recognized and lethal complication; however, incidence of venous thromboembolism has not been well-described. In this article, we described a 38-year-old Malay female patient admitted in 2008 with three-month history of peripheral neuropathy of lower limbs and right ankle ulcers. Initial inflammatory markers were high and perinuclear Anti-Neutrophil Cytoplasmic Antibodies were positive. She was diagnosed as anti-neutrophil cytoplasmic antibodies-associated vasculitis and started on intravenous methylprednisolone with methotrexate. She presented with relapse of skin vasculitis complicated with pulmonary renal syndrome after being stable for one year. She was intubated and proceeded with plasmapheresis and hemodialysis. She completed six cycles of cyclophosphamide. Renal biopsy revealed chronic changes consistent with end stage renal disease. She further relapsed in 2011 with nasal blockage, epistaxis, and nasal deviation. Chest X-ray revealed lung nodules. Prednisolone was increased, her symptoms settled, and she was discharged with azathioprine. She was readmitted at the end of the same year due to two-day history of right deep vein thrombosis and she later succumbed to methicillin-resistant Staphylococcus aureus sepsis.
A 17-year-old male student of Indonesian parentage presented with two weeks history of progressive painless bilateral visual deterioration. There was no contact with tuberculosis (TB)-infected patients and parents claimed that all immunization including BCG was completed. However, BCG scar was not apparent. Visual acuity was 6/36 and 6/60 in the right and left eyes respectively. The anterior and vitreous chambers were quiet. Funduscopic examination revealed retinal vasculitis with perivascular exudates, branch vein occlusion, neovascularization and macular oedema. Fluorescein angiography confirmed large areas of capillary non-perfusion and leaking new vessels. Mantoux test was positive and full regime anti-TB therapy was instituted. HIV screening was negative. Three days later, an immunosuppressive dose of oral steroid was started. Both eyes received intensive laser photocoagulation.Interestingly, there was no development of vitritis throughout.
Dengue fever is a common pyrexial-viral infection in the Asian region and the incidence is increasing yearly. There are various ocular manifestations of dengue fever such as maculopathy, retinal hemorrhages, retinal lesions and vasculitis. Reduction in visual acuity and other visual disturbances such as metamorphopsia is the most common presentation of dengue-associated maculopathy. We report an interesting case of a young lady with bilateral maculopathy secondary to dengue fever who was managed conservatively with dramatic visual improvements. This case highlights the possibility of bilateral and simultaneous ocular involvements in patients infected with dengue fever. Both ophthalmologists and physicians need to be aware of ocular complications following dengue fever and advice patients regarding the symptoms.
Children with Down syndrome have a higher risk of stroke. Similarly, intravenous immunoglobulin (IV Ig) is also known to cause a stroke. We reported a 3-year-old boy with Down syndrome who presented with severe pneumonia and received IV Ig. He developed right hemiparesis 60 hours after the infusion. Blood investigations, echocardiography and carotid Doppler did not suggest vasculitis, thrombophilia or extracranial dissection. Brain computerised tomography (CT) showed acute left frontal and parietal infarcts. Initial magnetic resonance angiography (MRA) of cerebral vessels showed short segment attenuations of both proximal middle cerebral arteries and reduction in the calibre of bilateral supraclinoid internal carotid arteries. The boy was treated with enoxaparin and aspirin. He only had partial recovery of the hemiparesis on follow-up. A repeat MRA 13 months later showed parenchymal collateral vessels suggestive of moyamoya disease. We recommend imaging the cerebral vessels in children with a high risk of moyamoya before giving IV Ig.
Childhood onset systemic lupus erythematosus is a rare disease that is more common amongst Southeast Asian children compared to the West. It is typified by a peripubertal onset and a female preponderance, which increases with advancing age. Organs commonly involved at diagnosis include haematological, renal, and mucocutaneous. Fever, malar rash, and cutaneous vasculitis are common. Lupus nephritis is typically proliferative especially Class IV and contributes to both disease activity and damage. Antinuclear antibody and anti-dsDNA positivity are both prevalent in this region. Disease activity is higher than Western cohorts at onset but responds to therapy reducing to low disease activity by six months. However, organ damage occurs early and continues to accumulate over the time, a consequence of both active disease (neurological and renal systems) and steroid-related complications especially in the eye (cataract and glaucoma) and musculoskeletal systems (avascular necrosis). Infections remain the leading cause of death and mortality in this region is highly variable contributed by the heterogeneity in social economic status, healthcare access, and availability of paediatric rheumatology expertise in the region.
The Nipah virus was first discovered in 1999, following a severe outbreak of viral encephalitis among pig farm workers in Malaysia. The virus was thought to have spread from Pteropus bats to pigs, then from infected pigs to humans by close contact. Mortality of the disease was high at about 40%. The main necropsy finding was disseminated microinfarction associated with vasculitis and direct neuronal involvement. Relapsed encephalitis was seen in approximately 10% of those who survived the initial illness. Since its first recorded emergence in peninsular Malaysia, 10 outbreaks of Nipah virus encephalitis have been reported in Bangladesh and West Bengal in India. The outbreaks occurred from January to May, with Pteropus giganteus as the reservoir of the virus. In Bangladesh, evidence indicated that the virus transmitted directly from bats to human, with human to human transmission as an important mode of spread. The mortality of the illness was higher in Bangladesh which stood at around 70%. This was likely to be due to genetic variation of the virus.
Tuberculous meningitis (TBM) causes significant morbidity and mortality. The primary objective was to re-examine the concept of "TB zone" and "ischaemic zone" in cerebral infarction in patients with tuberculous meningitis. The secondary objective was to evaluate cerebral infarction, vasculitis and vasospasm in tuberculous meningitis infections. Between 2009 and 2014, TBM patients were recruited. Neuroimaging was performed and findings of cerebral infarction, vasculitis and vasospasm were recorded. Infarcts were classified based on arterial supply and Hsieh's classification. Fifty-one TBM patients were recruited of whom 34 patients (67%) had cerebral infarction. Based on Hsieh's classification, 20 patients (59%) had infarcts in both "TB zone" and "ischaemic zones". 12 patients (35%) had infarcts in "ischaemic zone" and two (6%) patients had infarcts in "TB zone". In terms of vascular supply, almost all patients (35/36) had infarcts involving perforators and cortical branches. 25 patients (73%) and 14 patients (41%) had infarcts supplied by lateral lenticulostriate and medial lenticulostriate arteries respectively. 15 patients (37%) had vasculitis. Vasospasm was present in six patients (15%). 29 patients (85%) with cerebral infarction also had leptomeningeal enhancement (p = 0.002). In summary, infarcts involved mainly perforators and cortical branches, rather than "TB zone" versus "ischaemic zone".
Spontaneous coronary artery dissection is a rare event and commonly associated with pregnancy and female gender. This condition can reduce or completely obstruct the blood flow to the heart, causing a myocardial ischaemia, abnormalities in heart rhythm or sudden death. We present a case of a 28-year-old Indian male with no previous medical illness who complained sudden onset of chest pain prior to his death. Autopsy revealed a left anterior descending coronary artery dissection associated with plaque rupture. The anterior wall of left ventricle showed contraction band necrosis. There was also atheroma present in the right coronary artery which was insignificant. Histologically, dissection was associated with atherosclerosis. There was no evidence of vasculitis. The cause of death was given as coronary artery dissection due to coronary artery atherosclerosis.
Henoch-Schonlein purpura or anaphylactoid purpura is a systemic vasculitis of unknown cause that affects small vessels and mainly involves the skin, joints, gastrointestinal tract and kidneys. Gastrointestinal involvement occurs in more than half of patients and is thought to be related to edema and intramural haemorrhage. Radiologically the gastrointestinal findings are mainly those of bowel ischemia with thumbprinting and bowel wall oedema. Although this disease is usually treated conservatively, aggressive intervention is occasionally performed because of acute abdominal symptoms due to complications, such as perforation, intussusception and obstruction. This report illustrates a case of Henoch-Schonlein purpura with acute abdominal symptoms due to intussusception.
Objective: Pathological laughter, pseudobulbar affect or a myriad of its other synonyms, is a condition common secondary to neurological injury, presenting with uncontrolled laughing or crying, appearing to be mood-incongruent and significantly debilitating. The objective of this case report is to highlight a rare case of pathological laughter associated with Cerebral Lupus.
Methods: We report a case of a 27-year-old lady presenting with signs and symptoms of stroke with dysarthria progressing to aphasia and then pathological laughter, with an underlying poorly controlled Systemic Lupus Erythematosus, manifesting as Cerebral Lupus.
Results: An improvement in affect congruency followed by decreased frequency of outbreaks among bouts of pathological laughter.
Conclusion: The combination of Escitalopram and Topiramate is effective in the symptomatic treatment of Pathological Laughter.
Matched MeSH terms: Lupus Vasculitis, Central Nervous System
Management of inflammation after surgery for recalcitrant anterior uveitis is challenging. Herein, we report successful treatment using intracameral injection of recombinant tissue plasminogen activator (rtPA) in two patients with recalcitrant anterior uveitis, due to infective uveitis and Vogt-Koyanagi-Harada disease, respectively. A 40-year-old woman presented with bilateral redness and vision reduction that had persisted 2 weeks. She also had bilateral anterior uveitis, vasculitis, retinitis, and optic disc swelling. Serology was positive for Bartonella henselae and Toxoplasma gondii. She was treated using long-term systemic corticosteroids and appropriate antibiotics. Our second case; a healthy 30-year-old man with bilateral eye redness and reduced vision without pain, and associated with headache and tinnitus for 1 weeks. He showed bilateral granulomatous inflammation with vitritis, choroiditis, retinitis, and hyperemic optic disc. The patient was diagnosed with Vogt-Koyanagi-Harada disease and treated with systemic corticosteroids. Both patients developed secondary cataracts and glaucoma that necessitated surgical intervention. Persistent chronic inflammation led to the formation of a thick fibrin membrane anterior to the intraocular lens (IOL) after phacoemulsification surgery with IOL implantation. This membrane was removed surgically, and intracameral injection of rtPA (25 μg) was carried out. The persistent inflammation had resolved and visual acuity had significantly improved within 1 week of intracameral rtPA injection. There were no reported ocular or systemic side effects. Intracameral rtPA is beneficial in patients with recalcitrant anterior uveitis who have undergone intraocular surgery. In most cases, surgical intervention improves the patients' vision. Intracameral rtPA should be considered in cases of persistent inflammation of varying etiology.
A 71-year-old man with intestinal pseudo-obstruction was found to have a diffusely thickened adynamic small bowel with AA-amyloid in submucosal vessels and muscularis propria, foreign body giant cell reaction to amyloid, and necrotizing angiitis. The mucosa was unremarkable. Immunostains demonstrated numerous CD68+ monocyte/macrophages and CD8+ T cells associated with the amyloid deposits. The patient had no evidence of systemic vasculitis and no underlying cause for AA-amyloidosis was identified. Necrotizing angiitis coexistent with amyloid angiopathy has been reported in brain and temporal arteries, but not in the gastrointestinal tract and not with AA-amyloid. The inflammatory cell infiltrates in this case are consistent with a foreign-body and/or cell-mediated immunologic reaction to AA-amyloid, although a role for these cells in amyloid formation cannot be excluded.
Autoreactivity to myeloperoxidase (MPO) causes anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), with rapidly progressive glomerulonephritis. Here, we show that a Staphylococcus aureus peptide, homologous to an immunodominant MPO T-cell epitope (MPO409-428), can induce anti-MPO autoimmunity. The peptide (6PGD391-410) is part of a plasmid-encoded 6-phosphogluconate dehydrogenase found in some S. aureus strains. It induces anti-MPO T-cell autoimmunity and MPO-ANCA in mice, whereas related sequences do not. Mice immunized with 6PGD391-410, or with S. aureus containing a plasmid expressing 6PGD391-410, develop glomerulonephritis when MPO is deposited in glomeruli. The peptide induces anti-MPO autoreactivity in the context of three MHC class II allomorphs. Furthermore, we show that 6PGD391-410 is immunogenic in humans, as healthy human and AAV patient sera contain anti-6PGD and anti-6PGD391-410 antibodies. Therefore, our results support the idea that bacterial plasmids might have a function in autoimmune disease.
A 24-year-old Chinese gentleman presented with two weeks history of sudden floaters in his right temporal visual field associated with blurring of vision. This ex-smoker also reported chronic, bloody cough for two years and recent pleuritic chest pain. Examination revealed a thin patient with right eye visual acuity of 6/18 associated with optic nerve dysfunction, optic disc swelling and macula star, retinal vasculitis and retinitis. Despite anti-tuberculosis medication and corticosteroids, he developed neovascularisation. Subsequent vitreous haemorrhage necessitated trans pars plana vitrectomy, membrane peeling, endolaser and silicone oil injection. Final visual acuity was 6/9 with quiescent retinopathy.
Henoch – Schonlein purpura (HSP) is a vasculitis of small sized blood vessels. It is the most common acute systemic vasculitis in childhood. It mainly affects skin, gastrointestinal tract, joints and kidney. It results from Immunoglobulin A mediated inflammation. The characteristic skin findings are palpable purpuric lesions over the lower limbs and buttocks. The occurance of haemorrhagic bullae in children with HSP is rarely encountered. This case report describes a 12 year old boy with bullous haemorrhagic HSP treated at Institute Paediatric, Hospital Kuala Lumpur.
Typhoid fever is a systemic infection caused by Salmonella typhi, which may be associated with extra-intestinal
complications. Neurological manifestations, particularly Parkinsonism, are rarely reported. We report a
17-year-old patient with relapsed culture-proven Salmonella typhi infection who developed septic shock and
subsequently Parkinsonism. Lumbar puncture revealed acellular cerebrospinal fluid with raised protein level.
Magnetic resonance imaging revealed cerebral petechial haemorrhages resulted from small vessels vasculitis.
His symptoms resolved spontaneously after 3 months.
The presence of retinal vasculitis in patients with pulmonary tuberculosis is not uncommon. However, asymptomatic presentations are quite rare. Here, we present a case of 25-year-old Malay gentleman with pulmonary tuberculosis, who was referred for an eye assessment following initiation of anti-tuberculosis drugs. His vision was good; he had no other symptoms despite having retinal neovascularization, pre-retinal haemorrhages and healed choroiditis at the fundus of the left eye. Fundus fluorescein angiography showed multiple areas of capillary fallouts at the superotemporal quadrant of the left eye with leakage from the retinal neovascularization. He was treated with laser retinal photocoagulation and oral steroids for 6 weeks, while the anti-tuberculosis drugs were continued for 9 months. Subsequent follow-ups showed regression of the neovascularization. He has remained asymptomatic since his initial visit to the eye clinic.