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Background: Critically ill COVID-19 patients have an elevated risk of experiencing hypercoagulable conditions. Currently, many COVID-19 patients have been administered anticoagulation or antiplatelet therapies to lower the risk of systematic thrombosis. Iliopsoas hematoma is a potentially fatal and rare complication of bleeding disorders or anticoagulation therapy which sometimes grows to become clinically significant. The main purpose of this case review is to emphasize the importance of diagnosing iliopsoas hematomas and the possibility of antiplatelet contribution to its development. Case Presentation: We are reporting a rare presentation of non-traumatic iliopsoas hematoma in a non-anticoagulated patient. The patient is a 59-year-old male, with known type-2 diabetes, on oral hypoglycemic medications, 3-weeks post-COVID-19. He had started aspirin 81 mg orally, once daily, to prevent thrombotic events associated with COVID 19 infection, with no anticoagulant use and no other medications. He came in through the ED, presenting with two weeks history of progressive right lower limb weakness in which an iliopsoas hematoma diagnosis was confirmed based on radiological investigation. Conclusion: The possibility of iliopsoas hematoma should be considered in non-anticoagulated patients with no inherited or acquired coagulation disorders presenting with limb weakness. The link between antiplatelet use in a COVID-19 patient and the development of soft tissue bleeding (e.g., iliopsoas hematoma) must be studied further. © 2022 [The Author/The Authors]
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Background/Purpose: IgA vasculitis is the most common vasculitis affecting children. Vasculitis can be associated with the inflammatory process following infections, involving single or multiple organs. COVID-19 associated vasculitides have been reported variously, mostly Kawasaki-like features, livedo reticularis and rarely cutaneous small vessels vasculitis. Recently, there have been reports of IgA vasculitis following COVID-19 infection in children, although data among Asians are scarce. Method(s): Case report Results: We herein report a case of a previously healthy 6-year- old Thai boy with history of COVID-19 infection 4 weeks earlier, with only mild upper respiratory tract symptoms treated by a 5 day-course of favipiravir and supportive medication. He presented with rash over both lower limbs with difficulty to bear weight for a week. He denied fever, abdominal pain, nausea, vomiting, or any abnormal urinary symptoms. Physical examination revealed palpable purpura distributed on both lower legs with pain in his left foot and difficulty in bearing weight. His blood pressure was unremarkable for age at 97/67 mmHg. The initial investigations showed complete blood count with white cell count of 8.9 x 103/muL (neutrophils 47.3% and lymphocytes 42.4%), hemoglobin of 13.6 g/dL, which had no anemia for his age and platelet count of 297 x 103/muL. His urinalysis showed 2-3 red blood cells and 0-1 white blood cells per high power field without proteinuria and normal renal function. The erythrocyte sediment rate was 11 mm/hr and c-reactive protein was 3.9 mg/L, which were in normal range. He was diagnosed as IgA vasculitis and non-steroidal anti-inflammatory drug was prescribed to alleviate arthralgia of left foot. A week later, he revisited due to pain and swelling at his left scrotum. He was diagnosed as orchitis, one of the clinical manifestations of IgA vasculitis that can occur in boys. He had ongoing palpable purpura on the legs but pain at the left foot subsided. He then received oral prednisolone for the indication of orchitis at the dosage of 1 mg/kg/day with subsequent tapering for total duration of 3 weeks. All of his symptoms completely resolved. Conclusion(s): We present the interesting case of a Thai boy clinically diagnosed with IgA vasculitis following COVID-19 infection, having the clinical manifestations of palpable purpura, arthralgia, and orchitis. There are very limited data about post COVID-19- associated IgA vasculitis in children, especially in the Asian population. We would like to highlight this condition for physicians and to raise the awareness in the COVID-19 era.
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Purpose: To report a case of a 51-year- old male who developed dermatomyositis following the second dose of coronavirus disease (COVID-19) vaccine. Method(s): Case report Result: Case: We report a case of 51-year- old male who developed erythematous maculopapular rash on the upper anterior chest and upper back along with symmetric proximal muscle weakness two months after receiving his second dose of CoronaVac vaccine. His symptoms were followed by edema in the periorbital area which later involved the upper and lower extremities. He had dysphagia and weight loss. He had no known family history of autoimmune diseases. Physical examination revealed macular erythema over the lower anterior neck and upper back. Heliotrope rash and hyperkeratotic pink scaly papules on bilateral lateral second digits (mechanic's hands) were seen. Symmetric proximal muscle weakness in the upper and lower extremities was objectified. Blood tests showed elevated muscle enzymes (total CK: 3899 U/L, CK MB mass: 15.4 ng/mL, LDH: 683, AST: 232 U/L, ALT: 66 IU/L) elevated ESR (36) and normal CRP. Anti Jo 1 and anti U1 RNP were negative. Work up for systemic infection, thyroid function and malignancy were unremarkable. Diagnosis: Diagnosis of dermatomyositis was made based on clinical history and physical exam findings of symmetric proximal weakness, presence of heliotrope rash, V sign and shawl sign. Laboratory tests revealed elevated total CK, CK MB mass, LDH, AST, ALT and ESR consistent with an inflammatory myositis. Intervention(s): Hydrocortisone 1 mg/kg/day was started. Azathioprine was commenced on the 3rd hospital day. Ethical consideration: Informed consent for both written and photographic content was secured and patient confidentiality was observed. Conclusion(s): This case highlights the possible association between COVID 19 vaccine and this rare autoimmune disease. We hypothesize that among patients with genetic predisposition, the possibility of vaccines triggering and unmasking an autoimmune event is possible. (Figure Presented).
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Case Report: Acute transverse myelitis (TM) is a rare inflammatory disease that typically presents asweakness, sensory alterations, and bowel or bladder dysfunction. Among the causes of TM are infections, paraneoplastic syndromes, or autoimmune conditions of CNS. Postinfectious TM can develop secondary to a viral or bacterial infection. SARS-CoV-2 is a recently discovered viral illness, and sequelae due to COVID-19 infection are still being studied. There is scarce literature relating the two conditions, and it is imperative to raise awareness. A 72-year-old man with hypertension and GERD, completely independent in ADL, was brought to the ED with sudden onset of bilateral lower extremity weakness. He reported symptoms started with difficulty climbing stairs that rapidly progressed to inability to ambulate independently and were associated with bilateral thigh soreness. Nine days prior, he developed fever and generalized malaise, and two days later, SARS-CoV-2 PCR and Ag tests were positive. He received azithromycin, Paxlovid, and dexamethasone as treatment. Upon evaluation, the patient was afebrile and hemodynamically stable. Neurological examination was remarkable for spasticity and hyperreflexia at bilateral lower limbs, clonus, preserved motor strength with adequate sensation to soft touch, and intact vibration and proprioception in all extremities. Cranial nerves were intact. These findings were consistent with an upper motor neuron lesion. On imaging, the Head CT scan was unremarkable. Thoracic/Lumbar Spine MRI was significant for distal thoracic and conus areas with central homogeneous brightness compatible with nonspecific myelitis. Laboratories showed leukocytosis without neutrophilia or bandemia, thrombocytosis, and elevated CRP. HIV and RPR tests were negative. A lumbar puncture for CSF analysiswas remarkable for mild monocytic pleocytosis (7 cell/muL), an increased level of total proteins (56 mg/dL), and normal glucose (57 mg/dL). CSF culture and gram stain were negative. CSF cytology yielded few lymphocytes and few monocytes and was negative for malignant cells. The meningoencephalitis panel was negative. Based on these findings, a clinical diagnosis of postinfectious myelitis secondary to COVID-19was made. The patient was treated with intravenous Methylprednisolone 1 g daily for five days. On follow-up, lower extremity weakness resolved completely, and he resumed his daily physical activities. Patients with COVID-19 infection can present with neurologic manifestations such as headache, myalgias, dizziness, dysgeusia, and anosmia. This case hopes to raise awareness of less commonly known neurological manifestations of SARS-CoV-2 infection and how the early recognition of symptoms can help expedite the diagnosis and treatment of the condition to avoid long-term sequelae. [Figure presented] Copyright © 2023 Southern Society for Clinical Investigation.
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Introduction: An unusual case presentation of MOG-positive Acute Disseminated Encephalomyelitis (ADEM) in a preschool child following meta-pneumo viral infection responded to the combination of immune modulatory treatment with a favourable outcome. Material: Three-year-old female child presented with acute encephalopathy, high fever, vomiting, starring episodes, floppiness, and left abducent nerve palsy with rapid deteriorating GCS necessitating intubation and ventilation. Two weeks earlier, she was treated for suspected CNS infection with 10 days of antibiotics in the PICU with a positive meta-pneumo-virus. On admission, she had a GCS score of 6 with left-sided increased tone, bilateral hyperreflexia, and bilateral extensor response and on Day 14 demonstrated hyperkinetic movements of the upper and lower limbs. Method(s): Serum MOG antibody positive, CSF MOG low positive, metabolic investigations, Mycoplasma, EBV, Influenza, corona PCR, SARS-COV-2 Antibody, viral CSF panel unremarkable. MRI brain demonstrated T2 hyperintense signal in bilateral medial thalami and brain stem with a normal spine. Progressive changes were shown on repeated MRI Brains on day 4 and day 14 suggestive of multifocal changes involving deep cortical and subcortical white matter bilaterally with a new short segment of the spinal lesion at the T8 level. Repeated EEG and ambulatory EEG showed a diffusely slow background with intermittent slow runs of slow waves suggestive of generalized cerebral dysfunction. Result(s): After receiving the combination of high pulse steroids with a taper over 10 weeks, IVIG and 10 cycles of plasmapheresis she demonstrated gradual and remarkable clinical improvement over 10-12 weeks with a minimal focal neurological deficit. Conclusion(s): Initial differentiating CNS infection, metabolic disease and ADEM may be clinically challenging. Her clinical presentation, investigations and imaging were in keeping with the diagnosis of MOG-positive ADEM. Previous CNS infection may be related. MOG-positive ADEM treated with the early combination of immunomodulation may lead to positive clinical outcomes.
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Case report - Introduction: Bacterial community-acquired atypical pneumonia is sometimes complicated by myositis or by renal parenchymal disease. They can present with myositis and present with muscle weakness, pain or swelling, and elevated muscle enzymes. We present the case of a patient with lower limb weakness and raised creatinine kinase with atypical pneumonia caused by Legionella pneumophila. Case report - Case description: A 76-year-old Caucasian man, who was previously fit and independent and walked 3 miles every day presented with a 1-week history of progressive leg weakness, and inability to mobilize. He had a fall and was on the floor for 2 hours. He had a background history of hypercholesterolemia and was on atorvastatin for 15 years. On his vital observation, he was found tachypnoeic, tachycardic, and hypoxic. He had a right upper lobe crackle but he didn't have respiratory symptoms. His muscle power in his leg was 3/5 with carpet burns on knees and elbow. Initial investigation showed raised inflammatory marker CRP 412mg/L, AKI stage 1, and CK 43400 IU/L. His CXR showed dense right upper lobe consolidation. Legionella urinary antigen was positive. Myositis myoblot, ANA, ANCA negative. COVID-19 swab negative. Treated with IV antibiotic, supplemental oxygen, and IV fluid. Transferred to ITU due to worsening of hypoxia and kidney function. Interestingly, the CK level had improved significantly within 48 hours along with clinical improvement in his symptoms. There was no role of steroid or immunosuppressant due to his significant clinical improvement. On day 7 he was off oxygen, kidney function improved, had physiotherapy, and transferred to ward and on day 10 he was ambulant and discharged home. Case report - Discussion: To date, very few case reports of myositis in a patient with atypical pneumonia have been reported. The mechanism underlying acute myositis in atypical pneumonia is still unknown. The present analysis points out that the organism underlying atypical bacterial pneumonia may occasionally invade the muscle tissue thereby inducing both myositis and secondary kidney damage. Case report - Key learning points: We should be aware of this rare complication of atypical pneumonia and the resolution of symptoms that occur with the treatment of pneumonia. This would avoid unnecessary investigation and use of steroid.
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Purpose of Study: Report a rare case of onset of seronegative, juvenile dermatomyositis likely potentiated by Covid-19 infection Methods Used: Case analysis and literature research Summary of Results: A 7 year-old previously healthy male presented with 3 weeks of progressive, bilateral upper and lower extremity weakness, difficulty swallowing, voice changes, periorbital edema, and rash. Recent history was notable for diagnoses of COVID-19 one month prior to presentation and streptococcal pharyngitis 2 months prior to presentation. Notably, there is a family history of systemic lupus erythematosus. On examination, the patient demonstrated bilateral periorbital swelling with purple discoloration of the upper eyelids, a violaceous, pruritic, macular rash on his upper extremities and on his abdomen. Musculoskeletal exam was significant for severe axial (strength 2/5) and proximal (strength 3/5) muscleweakness with notable inability to sit unsupported or maintain head control. His neurologic exam was nonfocal;however, diffuse hyporeflexia in both upper and lower extremities were elicited. Initial screening labs were notable for mild transaminitis;positive ANA (1:80 in speckled pattern), negative ANCA, negative dsDNA/Anti- Sm, elevated aldolase of 10.3, CK 464, and LDH 665;normal thyroid studies and normal inflammatory markers. MRI with and without contrast of the spine indicated diffuse myositis of all muscle groups. Due to concern for autoimmune mediated myositis, Rheumatology was involved early in the patient's course. Empiric treatment was initiated early in the patient's presentation with IVIG, steroids, methotrexate, and plaquenil leading to gradual improvement in symptoms. Subsequent muscle biopsy was consistent with juvenile dermatomyositis (JDM). Conclusion(s): JDM is rare, occurring in 1 to 15 per million children. It classically presents with proximal myopathy and dermatologic findings of Gattron's papules, a heliotrope and malar rash. Its pathophysiology is not yet well defined but is thought to be a humoral mediated autoimmune disease. Muscle biopsies characteristically show perifascicular and perivascular infiltration. Early diagnosis and treatment with steroids, immune modulators, and physical therapy is critical to limit muscle atrophy. Viral infections are known triggers of rheumatologic diseases broadly;however, the more pronounced type 1 interferon response associated with COVID-19, which is known to be a driving pathway of JDM, may be a risk factor for severe, recalcitrant disease. Future research is needed to better identify involved pathophysiology and target future treatment efforts. Additionally, more education and case reports could focus on dermatologic presentations of individuals with pigmented skin. Copyright © 2023 Southern Society for Clinical Investigation.
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Background: Amyopathic dermatomyositis is a rare autoimmune skin disease with similar cutaneous manifestations as classic dermatomyositis (CDM), but with absent or subclinical muscle involvement i.e. Clinically Amyopathic Dermatomyositis (CADM). Inciting agents including vaccines have been linked to CADM. We describe a case of new-onset CADM associated with COVID-19 vaccine. Objective(s): To illustrate the occurrence of new-onset CADM in a 27-year- old male following COVID-19 immunization. Case Summary: A previously well 27-year- old male developed joint and muscle aches accompanied by scattered erythematous patches on the face and both upper and lower extremities, a week after receiving first dose of viral vector SARS-CoV- 2 vaccine. He took an anti-histamine and paracetamol with some relief of pains and slight clearing of the rashes. He proceeded to receive a second dose of the same vaccine 2 months later. A few days following the second dose, there was exacerbation of the skin lesions and was referred to Rheumatology clinics. Physical exam disclosed an ambulatory well-built male with normal vital signs and no objective muscle weakness. Skin involvement included facial rash, and the characteristic Heliotrope rash, Gottron's papules, and Holster sign. Complete blood counts, chemistries and muscle enzymes were within normal. Antinuclear antibody (ANA), erythrocyte sedimentation rate (ESR) and Smith/ribonucleoprotein (Sm/RNP) antibody were positive. He was managed with tapering prednisone and maintained on methotrexate and folic acid with significant improvement at time of this report. Conclusion(s): This is the first reported case of adverse reaction to COVID-19 vaccine that had studied in detail the skin and systemic autoimmune reaction. Development of autoimmune reaction following SARS-CoV- 2 vaccine has been described extensively;however, evidence of autoimmunity following vaccination is still relatively scant. Our case suggests that in predisposed subjects' vaccination could trigger an autoimmune reaction similar to the natural infection.
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Case Report: Initial History/Presentation: A term vaccinated 7-month-old male with a history of eczema presents with two hours of right-sided hemiplegia and hemidystonia. Parents deny loss of consciousness, altered mental status, or facial symptoms. He has no known history of recent or remote head trauma. Patient may have had COVID two months prior when he had upper respiratory symptoms, with his mother testing COVID+ at that time. Of note, he received a Moderna COVID vaccination one day prior to onset of symptoms. Physical Exam: Pertinent exam findings include CN II-XII intact, right-sided upper and lower extremity strength 3/5, sensation intact, and truncal ataxia while seated. Physical exam is otherwise unremarkable. Diagnostic Evaluation: Initial lab work revealed leukocytosis (20.9), but otherwise a reassuring CMP, triglycerides, HDL, and LDL. PTT was elevated, but normal on recheck. Protein C antigen and activity were low, but deemed non-concerning by hematology. All other hypercoagulable labs were normal. On imaging, CT Brain showed linear calcifications in bilateral basal ganglia suggestive of mineralizing angiopathy. HisCTA head/neckwas negative.MRI Brain revealed an acute infarct of the body/tail of the left caudate nucleus, posterior limb of internal capsule, and posterior putamen. Clinical Course/Follow-up: Our patient was started on Aspirin 4 mg/kg daily. Throughout the course of his 3-day inpatient stay, he had mild improvement of right-sided strength and function, and continued improvement upon follow-up with his pediatrician. Given the short interval between receiving his COVID vaccination and onset of symptoms, his case was reported to the Vaccine Adverse Event Reporting System. Conclusion(s): From a radiological perspective, mineralizing angiopathy is an uncommon but familiar finding seen in up to 5% of all neonatal head ultrasounds and increasing to nearly 20% in preterm infants. It is most commonly associated with infection, hypoxia, and chromosomal abnormalities but is usually of minimal clinical significance. However, there are numerous reports of basal ganglia and thalamic strokes following minor head trauma in children with mineralizing angiopathy. For radiologists, this association is important to recognize and relay to the primary team so targeted history and MRI, if indicated, may be obtained to expedite definitive diagnosis and initiation of treatment to preserve precious brain tissue. Without a history of head trauma, in this case, stroke provocation is unclear, and other infectious or inflammatory disorders could appear similarly if they induced vasospasm or blood pressure lability. A short-interval timeframe between COVID vaccine administration and symptom onset is likely incidental, but research to exclude or illicit any link may be of benefit. Findings of mineralizing angiopathy on CT in the appropriate clinical setting should prompt further evaluation with emergent MRI to determine the presence of basal ganglia or thalamic stroke. Copyright © 2023 Southern Society for Clinical Investigation.
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Case Report: A 46-year-old lady with medical history of hypertension, diabetes mellitus, and peripheral neuropathy was admitted to the hospital with the diagnosis of sepsis without septic shock secondary to a right foot pressure ulcer. Her presentation was non-specific as she was complaining of fatigue, myalgia, fever, and chills. Routine COVID-19 test was done on admission and it came back positive despite her denying having any respiratory symptoms. She was being treated with fluids and antibiotics until her third night of admission. According to the patient, she got up to use the restroom when she suddenly noticed weakness in her lower extremities. She thought it may be due to a muscle spasm;hence, she did not notify the medical team. Later, her nurse was routinely checking the patient's blood pressure when she noted a blood pressure of 220/105 mmHg. She then received a total of 3 doses of intravenous labetalol over three hours;however, her blood pressure continued to be elevated. Patient did endorse right flank pain but it was responding to intravenous fentanyl. The on-call physician then proceeded to perform a full physical examination and noted paleness, weakness, and absent pulses in bilateral lower extremities. A STAT computed tomography (CT) angiogram of the abdominal aorta and iliofemoral arteries was performed and it revealed low-density defects in the right kidney compatible with infarcts, occlusive thrombus in the infrarenal abdominal aorta and extensive bilateral arterial thrombosis. Vascular surgery was immediately consulted and they kept the patient on heparin drip and took her to the operation room within few hours for thrombectomies. Her blood pressure improved following the removal of the thrombus and there were no other documented occurrence of uncontrolled hypertension during her hospitalization. Discussion(s): Acute renal infarction is an arterial vascular event that leads to sudden disruption of blood flow in the renal artery. It can often be diagnosed late due to its rare incidence. In addition, it has a nonspecific clinical presentation that can mimic many common causes. The most common causes of renal infarction include atrial fibrillation, endocarditis, ischemic heart disease, hypercoagulable disorders, and spontaneous renal artery dissection. A case report published by Bourgault M, et al. on renal infarction suggested that around 97% had abdominal/flank pain and 48% of patients had marked uncontrolled hypertension at initial presentation of renal infarction. Our patient did not have any of the afore mentioned risk factors except for a possible hypercoagulable state from her COVID-19 infection and she did present with the two most common presentations. In conclusion, clinicians should have a low threshold for the suspicion of renal ischemia in patients with severe hypertension and flank pain. Copyright © 2023 Southern Society for Clinical Investigation.
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Case Report: Protein losing enteropathy (PLE) occurs when proteins leak from the gastrointestinal (GI) system more rapidly than they are produced. Inflammation of the GI tract facilitates increased membrane permeability of gastric mucosa, leading to excess protein leakage. 1 PLE in children has been associated with CMV, rotavirus, COVID-19, HIV, C. difficile, and autoimmune diseases like Crohn's Disease. 2-6 Norovirus is a known cause of PLE in immunocompromised pediatric patients. 7-8 However, to our knowledge, there are no case reports about PLE precipitated by norovirus in immunocompetent pediatric patients. The purpose of this case report is to present a case of PLE precipitated by a norovirus infection in a 4- year-old previously healthy child. While the above gastrointestinal viruses have been proposed as precipitators for this disease, PLE precipitated by norovirus infection has not been well described. This case also highlights the importance of early diagnosis and management to avoid complications. Method(s): Our patient initially presented with two days of abdominal pain, diarrhea, emesis, reduced urine output, and swelling of the lower extremities. He was exposed to several sick family members-his sister had upper respiratory symptoms and his grandmother had gastrointestinal symptoms. Physical exam was notable for diminished breath sounds in the right lower lobe, abdominal distension with diffuse tenderness and dullness to percussion, significant scrotal and penile edema, and bilateral lower extremity pitting edema. Laboratory results revealed leukocytosis, hypoalbuminemia, hyponatremia, elevated aspartate aminotransferase (AST), and elevated serum alpha-1-antitrypsin, as well as low Immunoglobulins G and M. CD3 and CD4 levels were low reflecting cellular immune dysregulation seen in patients with PLE. IgA and Tissue Transglutaminase (TTF) were within normal limits. Ebstein Barr Virus and cytomegalovirus IgM antibodies were negative. COVID IgG was negative as well. His Polymerase chain reaction (PCR) gastrointestinal panel was positive for norovirus. A chest X-ray showed a large right pleural effusion. Abdominal CT revealed large ascites slightly more predominant in the upper abdomen, mesenteric lymphadenitis, and bilateral pleural effusions. Echocardiogram showed small anterior and apical pericardial effusions. Result(s): Based on the patient's elevated serum alpha-1 antitrypsin levels, hypoalbuminemia, low levels of immunoglobulins and lymphocytes, and clinical manifestations of ascites, bilateral pleural effusions, pericardial effusion, and dependent edema, along with a positive PCR for norovirus, the diagnosis of PLE secondary to Norovirus was made. Conclusion(s): This case demonstrates the importance of recognizing viruses like Norovirus as potential causes of PLE to avoid a delay in diagnosis and initiation of therapy, and to avoid unnecessary additional testing. Copyright © 2023 Southern Society for Clinical Investigation.
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Introduction/Background: Antiphospholipid syndrome (APS) is a rare autoimmune multisystem disease characterised by thrombosis and pregnancy morbidity in the presence of persistently elevated titres of: lupus anticoagulant, anticardiolipin and/or anti-glycoprotein 1. It may be primary (occurring alone) or secondary (in combination with another disease, most commonly systemic lupus erythematosus (SLE)). Recent publications highlighted clinical criteria limitations for children and raised awareness of the burden and prevalence of non-criteria manifestations in this population. This case report adds further weight to the need to raise multi-specialty awareness of non-criteria manifestations to aid recognition and treatment of this rare condition with potentially severe sequelae. Description/Method: 13-year-old female with SLE diagnosed aged 8 in India with bilateral optic neuritis occurring two months later. ANA positive at diagnosis with low complement and thrombocytopenia. Treated with prednisolone and hydroxychloroquine. Patient moved to the UK aged 9;initial abnormal bloods: mildly positive ANA (ENA negative), thrombocytopenia, strong lupus anticoagulant. As serology not strongly suggestive and optic neuritis rare in lupus diagnosis questioned. Ophthalmology review confirmed bilateral optic atrophy without evidence of previous vasculitis. There was debate whether the postretinal demyelination was due to antiphospholipid syndrome or a primary demyelinating condition. Hydroxychloroquine stopped and azathioprine started. Following normal neurology investigations (brain, spine MRI/MRV/MRA) concluded if patient developed new APSrelated symptoms or worsening visual evoked potentials anticoagulation would be discussed. Patient remained stable over four years with chronic thrombocytopenia and ESR persistently elevated. Azathioprine changed to Mycophenolate mofetil (MMF) due to side effects. Routine medication monitoring bloods in 2022 showed ESR 97, CRP 78, Platelets 61. Review identified vasculitic rash on soles of both feet with palpable nodules and normal pulses. Further investigation confirmed antiphospholipid antibody triple positivity. Aspirin commenced, hydroxychloroquine restarted, MMF dose increased and rituximab administered. Left foot rash settled but right progressed with toe discolouration and numbness. Skin biopsy considered but not performed due to skin integrity concerns. Foot pulses remained present and normal. Bilateral lower limb doppler reported as normal;increased symptoms resulted in CT angiogram which revealed bilateral non-occlusive popliteal thrombus and left pulmonary embolus. Subsequent echocardiogram was normal. Patient was anticoagulated with low molecular weight heparin followed by warfarin. Vascular surgical team advocated medical management and patient received seven infusions of Iloprost followed by Sildenafil. She achieved near total resolution of skin changes to toes with only minimal loss of skin over tip of right great toe. Patient will now require long-termanticoagulation. Discussion/Results: APS was considered in initial differential diagnosis but patient did not meet current clinical criteria as no past evidence of thrombosis. Lupus anticoagulant was consistently strongly positive and anticardiolipin repeatedly negative. As anti-B2 glycoprotein 1 antibody is not routinely tested and must be verbally requested, it was only checked once (negative) prior to discovery of triple positivity. ANA reported as strongly positive at time of SLE diagnosis but reviewing original notes from India titre was 1:100 and therefore not highly convincing. ENA negative and complement and white cell count normal on repeat testing since. Therefore, it is probable that this patient has primary APS as opposed to secondary APS in association with SLE. However, it is possible that this patient may develop more symptoms of SLE over time. When this patient presented with foot rash there were high numbers of children presenting with varying severity of painful, itchy toes coined 'covid toes' due to suspected lin to SARS-CoV-2 infection. Patient had exposure history, and COVID antibody serology was difficult to interpret due to recent vaccination. Dermatology found appearance to be consistent with 'covid toes' and advised supportive treatment. The triple APS antibody positivity result provided probable aetiology. Providing evidence of thrombus was problematic with false reassurance from apparently normal lower limb arterial doppler when actually popliteal arteries were not checked in view of the presence of normal flow proximally at the groin and distally in the feet. This case highlights the need to continue to search for thrombus in presence of high titres antiphospholipid antibodies and particularly in the case of triple positivity as although patient presented with colour change to toes, she was entirely asymptomatic from her PE and her left foot improved spontaneously despite a left popliteal thrombus also being present. Key learning points/Conclusion: Non-criteria manifestation of thrombocytopenia (occurs in 25% paediatric APS patients) was present throughout and patient had past history of haematuria (a recognised renal non-criteria manifestation). A paediatric specific APS criteria including these may have resulted in earlier detection of triple antiphospholipid antibody positivity and thus earlier treatment escalation and possible avoidance of thrombus. It has been reported that a high proportion of children with positive antiphospholipid antibodies don't develop a thrombus. However, it is interesting that our patient was entirely asymptomatic from her pulmonary embolus which was an incidental finding on her CT angiogram. This prompts a discussion about how much imaging should be performed in those with high levels of persistent positive antiphospholipid antibodies. Rituximab resulted in normalisation of platelet count and ESR for the first time since initial presentation. Anticardiolipin antibodies normalised, lupus anticoagulant decreased from strong to moderate and anti- B2 glycoprotein levels decreased but remained positive. Rituximab is a recognised treatment for catastrophic antiphospholipid syndrome (CAPS) but not routinely used in APS. The consistently raised ESR in an apparently clinically well patient is a reminder to continue to search for causes of inflammation. As the CRP was largely in normal range, this demonstrates the unique value of the ESR. In view of anti-B2 glycoprotein 1 antibody requiring to be verbally requested, discussions are ongoing with the laboratory department regarding the possibility of electronic request and a comment with recommendation to check other two antiphospholipid antibodies following one positive antibody result. As a result of this case, a plan will be put in place to ensure annual screening of antiphospholipid antibodies in all juvenile SLE patients in our care. It is hoped that this case report promotes discussion amongst the paediatric rheumatology community regarding further research required for development of paediatric specific APS criteria and management.
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BACKGROUND: A decrease in the frequency of amputations due to diabetic foot syndrome (DFS) is one of the parameters that determine the quality of medical care for patients with diabetes mellitus. AIM: Our aim was to study the indicators characterizing medical care for patients with lower limb pathology in diabetes mellitus in St. Petersburg from 2010 to 2021. MATERIALS AND METHODS: Annual reports on the treatment of patients with DFS in city hospitals specializing in the surgical treatment of DFS and in outpatient offices <<Diabetic foot>> (DFO) from 2010 to 2021 were analyzed. RESULT(S): The average number of patients per year admitted to the DFO was 18,527 (34,440 visits). Proportion of patients with foot ulcers - 8,9%, with Charcot's arthropathy - less than 1%. Before 2020, the frequency of above the foot amputations decreased from 48.3% to 8.6%, hospital mortality - from 11.7 to 5.7%, the number of revascularizations increased from 37 to 642 per year. The increase in operational activity was not accompanied by a decrease in the frequency of amputations (59.3% in 2019). Of all amputations, 11.3% were patients referred from DFO. During the epidemic, the number of visits and patients admitted to the DFO decreased by 27,3% and 31%, respectively. The proportion of foot ulcers and the frequency of amputations have not changed. Inpatient care was characterized by a decrease in operational activity, a decrease in the availability of revascularization, a 2-fold increase in the proportion of high amputations and an increase in hospital mortality from 5.7% in 2019 to 14.9% in 2021. CONCLUSION(S): An analysis of the statistics of specialized care for patients with DFS over 12 years showed the reduction of the frequency of high amputations, but revealed an increase in the frequency of surgical interventions in DFS against the background of an almost unchanged proportion of amputations in the structure of all operations. Despite significant quantitative indicators, the outpatient service seems to be insufficiently effective in reaching the target population. The negative impact of the epidemic has led to a significant increase in the frequency of high amputations and mortality. Copyright © Endocrinology Research Centre, 2022.
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Introduction: Coronavirus disease 2019 (COVID-19) pandemic is ongoing and new variants of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV- 2) are emerging. There is an urgent need for COVID-19 vaccines to control disease outbreaks by herd immunity. Vaccines can trigger immunity as many vaccine-related immunological adverse events have been described. Case Presentation: A case of a 32 years old Filipino female with no known co-morbidity who presented with rashes on bilateral lower extremities one day after receiving her second dose of CoronaVac-Sinovac vaccine. These were non-pruritic nor painful, which appeared to be initially well-defined round erythematous macules, papules, and plaques, mostly raised. It was associated with colicky abdominal pain and inflammatory arthritis affecting the both knees and ankles. She has no vices but known to have allergy with seafood. There were multiple well defined erythematous round to irregularly shaped purpuric macules, papules and plaques, non-blanching, flat and raised, on arms near the antecubital fossa, abdomen and lower extremities. She underwent skin biopsy and direct immunofluorescence showed interface dermatitis with leukocytoclastic vasculitis and IgA +1 vessel wall, and fibrinogen +2 vessel wall, respectively. There was microscopic hematuria and proteinuria. The Urine protein creatinine ratio was normal at 0.193 gm/gm. She was managed as a case of IgA vasculitis and was given moderate dose of steroid (0.5mg per kilogram per day prednisone equivalent) and omeprazole. She was discharged improved with resolution of rashes evident during follow up at the out-patient consultation. Conclusion(s): We report a case of an adult Filipina developing IgA vasculitis following CoronaVac COVID-19 vaccination. She responded well following initiation of steroid therapy. Autoimmune phenomenon following immunization is possible through different mechanisms. These include molecular mimicry, a hyper-stimulated inflammatory state, and autoimmune syndromes induced by adjuvants. While no strategies have been found to prevent autoimmunity following vaccination, it should be emphasized that vaccine recipients should seek medical care for any untoward events following receipt of any immunization.
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Background/Purpose: Multisystem inflammatory syndrome in children (MIS-C), associated with COVID-19 infection is a life-threatening condition, required intensive care. The aim of this study was to determine risk factors for severe/life-threatening course of MIS-C. Method(s): The retrospective study included 166 children (99 male, 67 female), aged from 4 months to 17 years (median 8.2 years), who met the WHO criteria for MIS-C. The criterion of severity was the fact of the ICU admission. The analysis of the obtained data was performed using the STATISTICA software package, version 10.0 (StatSoft Inc., USA). Result(s): To assess the factors associated with the severe course of MIS-C, patients were divided into two groups: those who were hospitalized in the ICU (n = 84;50.6%), and those who did not (n = 82;49.4%). Patients with a more severe course of MIS-C were significantly older. They had a high frequency of signs such as rash, edema, hepatomegaly, splenomegaly, neurological and respiratory symptoms. Hypotension/shock and myocardial damage were much more common in patients hospitalized in the ICU. Among the laboratory changes there were significant differences in the levels of hemoglobin, leukocytes and platelets, CRP, creatinine, troponin and D-dimer. The presence of macrophage activation syndrome was higher in patients, admitted in the ICU. Children, required intensive care required high dose corticosteroids and IVIG more often (table 1). FIGURE: 1) The first symptoms of progeria in infancy: scleroderma-like changes in the skin of the lower extremities and stiffness of knee joints at the age of 2 months. 2) Girl at the age of 3 years 5 months. Almost total alopecia with the absence of eyebrows and eyelashes. Pronounced venous pattern in the forehead, nasal bridge and nasolabial triangle. Conclusion(s): MIS-C is potentially a severe life-threatening condition, in which more than half (50.6%) of patients needed the ICU admission. The main factors determining the severity of MIS-C were: cardiovascular, resiratory and central nervous system disorders. It has been found that factors such as hepatomegaly, splenomegaly, D-dimer >2568 ng/ml, troponin >10 pg/ml, make it possible to identify a group of patients with high risk of severe MIS-C who may potentially need hospitalization in the ICU.
ABSTRACT
Case Report: Acute motor and sensory axonal neuropathy (AMSAN) syndrome is a rare subtype of Guillain-Barre syndrome (GBS) with poor recovery [1]. While respiratory and gastrointestinal infections may precipitate AMSAN, an underlying autoimmune disorder is seldom reported in literature. We herein report the complex case of a patient with undiagnosed, asymptomatic mixed connective tissue disease (MCTD) who developed AMSAN syndrome. Case: A 44-year-old Asian male without medical history presented with progressively worsening weakness of both upper and lower extremities and inability to perform daily activities. His symptoms started 12 weeks prior with difficulty standing from a seated position. He felt subjectively better for some time until a week prior, when he became bedbound. He had diarrhea 6 months ago, with 5-6 loose bowel movements a day for a few weeks. Vital signs on admission was normal. On neurological examination, he was alert and oriented, with bilateral upper and lower extremity flaccid paralysis, diffuse muscle atrophy, bilateral hand and foot drop, negative Hoover sign, diffuse areflexia, and intact sensation. Cerebrospinal fluid (CSF) analysis showed WBC 0 and protein level 136. MRI cervical, thoracic, and lumbar spine were normal. EMG revealed sensory involvement with positive sharp waves in proximal muscles along with fibrillations. Intravenous immunoglobulin (IVIG) was initiated at 0.4 mg/kg for 5 days. Infectious workup for COVID-19, stool culture, HIV, TB, RPR and campylobacter jejuni antibody (Ab), was negative. ANA was positive in a speckled pattern with titres 1:1280, with a positive RNP Ab, SS-A, and RF IgM, IgG and IgA. Rest of the autoimmune workup (anti-dsDNA, anti-CCP, SS-B, aldolase, anti-Jo-1, anti-Scl-70, p-ANCA, c-ANCA, anti-GM1, anti-GQ1b, and anti-GD1a ganglioside Ab) was negative. The myositis specific 11 Ab panel was negative. Despite IVIG therapy, he developed dysphagia, respiratory distress, with a negative inspiratory force of -0, requiring intubation. He had a tracheostomy and PEG tube placed and remains quadraplegic nearly 120 days later. Discussion(s): The authors report a unique case of a patient who became progressively weak over 3 months, leading to complete quadriplegia. Interestingly, this is more consistent with chronic inflammatory demyelinating poly-neuropathy (CIDP), as AMSAN typically develops over a short period of 2 to 4 weeks [2]. Despite having negative anti-GM1 and anti-GD1a Ab (in which positive Ab are pathognomonic but not always present in AMSAN syndrome), the patient had weakness that began in the lower extremities, progressing to paralysis, along with albuminocytological dissociation on CSF analysis, pointing to a GBS diagnosis [3]. He had sensory involvement in the EMG, thus making the diagnosis as AMSAN. He had an undiagnosed, asymptomatic autoimmune process most consistent with MCTD. Whether the two disease processes are related to each other is a concept that has not yet been investigated. Pediatric Clinical Case Reports Concurrent Session Saturday February 4, 2023 1:00 PM Copyright © 2023 Southern Society for Clinical Investigation.
ABSTRACT
Serratia marcescens represents an unusual yet potentially deadly cause of lower limb necrotizing fasciitis (NF). Compounding the already high mortality of NF, S. marcescens infections are usually associated with worse outcomes (i.e., amputation). Here we present the case of a 56-year-old immunocompromised man due to lupus nephritis who developed lower limb NF secondary to S. marcescens followed by nosocomial coronavirus disease 2019 (COVID-19) pneumonitis. Successful limb salvage was achieved through a multidisciplinary team approach from various specialties including plastic surgery, orthopedic surgery, anesthesiology, intensive care, respiratory medicine, and nephrology. At 11 months' follow-up, the patient was largely independent with activities of daily living and was able to ambulate. Unfortunately, he suffered a myocardial infarction at 19 months post-operatively and passed away. A review of the literature revealed only a handful of cases of lower limb NF due to S. marcescens and none with subsequent COVID-19. Therefore, this is the first report of such a case which should help with the clinical management of such cases going forward, especially with COVID-19 now becoming endemic in our communities and contributing to delayed presentations and increased mortality in NF.