BACKGROUND

Spontaneous isolated superior mesenteric artery dissection (SISMAD) is a rare vascular condition where the superior mesenteric artery is affected by dissection without involving other arteries. Its incidence is estimated at 0.06% to 0.08% globally. The possible causes include polyarteritis nodosa (PAN), an autoimmune disease affecting medium-sized arteries. SISMAD can manifest with various clinical presentations, from asymptomatic to acute bowel ischemia, warranting consideration when common causes of acute abdomen are ruled out.

This is the case of a 44-year-old female Filipino, hypertensive, who complained of abdominal pain, initially managed as intestinal amoebiasis. Abdominal examination showed a soft, non-tender abdomen with no guarding, making the symptoms disproportionate to physical examination. Due to persistence of symptoms despite a full antibiotic course, further workup was done. Computed tomography (CT) scan of the whole abdomen with contrast revealed an isolated dissection of the proximal superior mesenteric artery (SMA) with thrombosis which was confirmed on CT angiography. The diagnosis of PAN was established based on the correlation of clinical presentation, laboratory findings and imaging results. Conservative management was done and to address the thrombosis, anticoagulation with heparin was initiated. The patient was also given methylprednisolone pulse therapy and cyclophosphamide with good response. Resolution of symptoms noted and she was eventually discharged improved.

SISMAD and PAN are independently rare conditions. This unique case involved both diseases in a 44-year-old Filipino female, and to date, there have been no reported similar cases worldwide. Early diagnosis of the disease requires a high degree of suspicion and pattern recognition. This is crucial for timely treatment and improved prognosis. Furthermore, close surveillance is important to identify potential relapses even after symptom resolution.

Spontaneous isolated superior mesenteric artery dissection (SISMAD) is a rare vascular condition where the superior mesenteric artery is affected by dissection without involving other arteries. Its incidence is estimated at 0.06% to 0.08% globally. Possible causes include trauma, congenital connective tissue disorders, arteriosclerosis, and vasculitis such as polyarteritis nodosa (PAN). SISMAD can manifest with various clinical presentations, from asymptomatic to acute bowel ischemia, warranting consideration when common causes of acute abdomen are ruled out.

This is a case of a 44-year-old female Filipino, who complained of abdominal pain, initially managed as intestinal amoebiasis. Abdominal examination showed a soft, non-tender abdomen with no guarding, making the symptoms disproportionate to physical examination. Due to persistence of symptoms despite full antibiotic course, further work up was done. Computed tomography (CT) scan of the whole abdomen with contrast revealed an isolated dissection of the proximal superior mesenteric artery (SMA) with thrombosis which was confirmed on CT angiography. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), cytoplasmic antineutrophilic cytoplasmic antibodies (ANCA) and perinuclear ANCA were both negative; antinuclear antibody (ANA), C3 and C4 levels were also unremarkable. The diagnosis of PAN was established based on the correlation of clinical presentation, laboratory findings, and imaging results. She was given methylprednisolone pulse therapy and cyclophosphamide with good response. Anticoagulation with heparin was initiated to address the thrombosis. The patient had complete bowel rest, pain management, intravenous hydration, and parenteral nutrition. Resolution of symptoms noted and was eventually discharged improved.

SISMAD and PAN are independently rare conditions. This unique case involved both diseases in a 44-year-old Filipino female. Early diagnosis of the disease requires a high degree of suspicion and pattern recognition. This is crucial for timely treatment and improved prognosis. Furthermore, close surveillance is important to identify potential relapses even after symptom resolution.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-system disease that presents significant diagnostic challenges due to its complexity and low incidence (White and Dubey, 2023). It affects males and females equally, though males may exhibit more active disease at diagnosis and often require more aggressive treatment (Liu et al., 2023). The hallmark features of EGPA include delayed-onset asthma, eosinophilia in tissues and blood, and vasculitis affecting small to medium-sized arteries (White and Dubey, 2023). EGPA falls under the category of antineutrophil cytoplasmic antibody (ANCA)‍-associated vasculitis (AAV), whereas only about half of EGPA patients test positive for ANCA (Khoury et al., 2023).
Humans ; Male ; Hemorrhage/etiology* ; Granulomatosis with Polyangiitis/complications* ; Heart Arrest/etiology* ; Early Diagnosis ; Eosinophilia/diagnosis* ; Kidney Diseases/etiology* ; Churg-Strauss Syndrome/complications* ; Middle Aged

INTRODUCTION

Childhood Polyarteritis Nodosa (PAN) is a rare, systemic necrotizing vasculitis of the small to medium-sized vessels with an uncertain global distribution. The primary etiology is unknown. However, PAN is commonly associated with preceding Group A streptococcus infection in children. The most common cutaneous manifestations of PAN include tender subcutaneous nodules, livedo reticularis, digital ischemia and ulceration. To date, no reports have documented cutaneous PAN as a sequela of rheumatic fever.

This is a report of a 7-year-old Filipino male who presented with multiple, well-defined erythematous to hyperpigmented, firm, tender nodules, with some areas of lace or net-like macules and patches, some resolved leaving hyperpigmentation measuring 1x1 cm to 2x2 cm on the lower back, bilateral upper and lower extremities accompanied by fever, malaise, arthralgia and myalgia with a previous history of rheumatic fever. A 6mm skin punch biopsy revealed findings consistent with PAN. The patient was managed with prednisone. Due to the limited response to treatment, he was eventually given mycophenolate mofetil.

Childhood polyarteritis nodosa (PAN) is a rare form of necrotizing inflammation of the medium-sized blood vessels primarily linked to Group A streptococcal infection in children, with no known reported cases associated with rheumatic fever. However, in this case, we were able to observe that PAN could present as a probable rare sequela of rheumatic fever. This warrants close follow-up among such patients.

Humans ; Lupus Vasculitis, Central Nervous System/pathology* ; Magnetic Resonance Imaging/methods* ; Diffusion Tensor Imaging/methods* ; Patients ; Lupus Erythematosus, Systemic/pathology* ; Brain/pathology*

Humans ; Child ; Voriconazole/adverse effects* ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy* ; Renal Dialysis ; Seizures ; Antibodies, Antineutrophil Cytoplasmic

Systemic lupus erythematosus (SLE) associated macrophage activation syndrome (MAS) is clinically severe, with a high mortality rate and rare neuropsychiatric symptoms. In the course of diagnosis and treatment, it is necessary to actively determine whether the neuropsychiatric symptoms in patients are caused by neuropsychiatric systemic lupus erythematosus (NPSLE) or macrophage activation syndrome. This paper retrospectively analyzed the clinical data of 2 cases of SLE associated MAS with neuropsychiatric lesions, Case 1: A 30-year-old female had obvious alopecia in 2019, accompanied by emaciation, fatigue and dry mouth. In March 2021, she felt weak legs and fell down, followed by fever and chills without obvious causes. After completing relevant examinations, she was diagnosed with SLE and given symptomatic treatments such as hormones and anti-infection, but the patient still had fever. The relevant examinations showed moderate anemia, elevated ferritin, elevated triglycerides, decreased NK cell activity, and a perforin positivity rate of 4.27%, which led to the diagnosis of "pre-hemophagocytic syndrome (HPS)". In May 2021, the patient showed mental trance and babble, and was diagnosed with "SLE-associated MAS"after completing relevant examinations. After treatment with methylprednisolone, anti-infection and psychotropic drugs, the patient's temperature was normal and mental symptoms improved. Case 2: A 30-year-old female patient developed butterfly erythema on both sides of the nose on her face and several erythema on her neck in June 2019, accompanied by alopecia, oral ulcers, and fever. She was diagnosed with "SLE" after completing relevant examinations, and her condition was relieved after treatment with methylprednisolone and human immunoglobulin. In October 2019, the patient showed apathy, no lethargy, and fever again, accompanied by dizziness and vomiting. The relevant examination indicated moderate anemia, decreased NK cell activity, elevated triglycerides, and elevated ferritin. The patient was considered to be diagnosed with "SLE, NPSLE, and SLE-associated MAS". After treatment with hormones, human immunoglobulin, anti-infection, rituximab (Mabthera), the patient's condition improved and was discharged from the hospital. After discharge, the patient regularly took methylprednisolone tablets (Medrol), and her psychiatric symptoms were still intermittent. In November 2019, she developed symptoms of fever, mania, and delirium, and later turned to an apathetic state, and was given methylprednisolone intravenous drip and olanzapine tablets (Zyprexa) orally. After the mental symptoms improved, she was treated with rituximab (Mabthera). Later, due to repeated infections, she was replaced with Belizumab (Benlysta), and she was recovered from her psychiatric anomalies in March 2021. Through the analysis of clinical symptoms, imaging examination, laboratory examination, treatment course and effect, it is speculated that the neuropsychiatric symptoms of case 1 are more likely to be caused by MAS, and that of case 2 is more likely to be caused by SLE. At present, there is no direct laboratory basis for the identification of the two neuropsychiatric symptoms. The etiology of neuropsychiatric symptoms can be determined by clinical manifestations, imaging manifestations, cerebrospinal fluid detection, and the patient's response to treatment. Early diagnosis is of great significance for guiding clinical treatment, monitoring the condition and judging the prognosis. The good prognosis of the two cases in this paper is closely related to the early diagnosis, treatment and intervention of the disease.
Humans ; Female ; Adult ; Rituximab/therapeutic use* ; Macrophage Activation Syndrome/etiology* ; Retrospective Studies ; Lupus Erythematosus, Systemic/drug therapy* ; Methylprednisolone/therapeutic use* ; Lupus Vasculitis, Central Nervous System ; Fever/drug therapy* ; Erythema/drug therapy* ; Hormones/therapeutic use* ; Anemia ; Alopecia/drug therapy* ; Triglycerides/therapeutic use* ; Ferritins/therapeutic use*

Both anti-glomerular basement membrane (GBM) disease and the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are common causes of pulmonary-renal syndrome. Organizing pneumonia (OP), a special pattern of interstitial lung disease, is extremely rare either in AAV or anti-GBM disease. We report an old woman presented with OP on a background of co-presentation with both ANCA and anti-GBM antibodies.
Female ; Humans ; Antibodies, Antineutrophil Cytoplasmic ; Organizing Pneumonia ; Autoantibodies ; Glomerulonephritis ; Anti-Glomerular Basement Membrane Disease ; Pneumonia ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications*

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) has a wide range of symptoms, and it is difficult for clinicians to make a quick and correct diagnosis. On November 11, 2021, a 36-year-old male patient with AAV was admitted to the emergency and critical care department of Yichang Central People's Hospital. He was admitted to the emergency intensive care unit (EICU) with gastrointestinal symptoms (abdominal pain, black stool) as the main physical signs, and was initially diagnosed as AAV with gastrointestinal hemorrhage (GIH). No bleeding point was found after repeated gastroscopy and colonoscopy. Abdominal emission CT (ECT) showed diffuse hemorrhage in the ileum, ascending colon and transverse colon. Multi-disciplinary consultation in the whole hospital considered the diffuse hemorrhage caused by small vascular lesions in the digestive tract caused by AAV. Pulse therapy with methylprednisolone 1 000 mg/d and immunosuppressive therapy with cyclophosphamide (CTX) 0.2 g/d were administered. The patient's symptoms quickly relieved and transferred out of the EICU. After 17 days of treatment, the patient finally died of massive gastrointestinal bleeding. A systematic review of relevant literatures combined with the case diagnosis and treatment process found that only a minority of AAV patients present with gastrointestinal symptoms as their first symptoms, and patients with GIH were very rare. Such patients had a poor prognosis. This patient delayed the use of induced remission and immunosuppressive agents due to the treatment of gastrointestinal bleeding, which may be the main cause of life-threatening GIH secondary to AAV. Gastrointestinal bleeding is a rare and fatal complication of vasculitis. Timely and effective induction and remission treatment is the key to survival. Whether patients should receive maintenance therapy, the duration of maintenance therapy, and the search for markers of disease diagnosis and treatment response are directions and challenges for further research.
Male ; Humans ; Adult ; Gastrointestinal Hemorrhage ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Critical Care ; Cyclophosphamide ; Death

OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis, which often starts with respiratory symptoms such as asthma, and it is difficult to make early clinical diagnosis.This study aims to improve the therapeutic level of EGPA with lung involvement via analyzing the clinical characteristics, diagnosis, and treatment . METHODS: We retrospectively analyzed the clinical data of 13 EGPA patients with lung involvement who were diagnosed from February 1, 2014 to July 31, 2021 in the Second Xiangya Hospital, Central South University. RESULTS: The ratio of male to female in 13 patients was 7꞉6. The patients were diagnosed at median age 52 (46-68) years old and 6 had been diagnosed as "bronchial asthma". Pulmonary clinical manifestations mainly included cough, expectoration, wheezing, and shortness of breath; while extra-pulmonary manifestations mainly included rash and subcutaneous mass, fever, limb numbness, muscle and joint pain, abdominal pain, etc. Peripheral blood tests of all patients showed that 11 patients had eosinophils ≥10%, 10 had elevated inflammatory indicators, and 3 were anti-neutrophil cytoplasmic antibody (ANCA) positive. The major lung imaging features were patches or strips of increased density, multiple nodules, bronchiectasis, bronchial wall thickening, exudation, mediastinal lymph nodes, and so on. Eight patients had sinusitis and 9 with abnormal electromyography. Extravascular eosinophil infiltration was found in 9 patients. Six patients with lung biopsy showed eosinophil, lymphocyte, and plasma cell infiltration, 3 patients were involved in small blood vessels, and 1 had granuloma. Pulmonary function tests were performed in 7 patients, 5 of them showed different degrees of pulmonary ventilation dysfunction, and 4 of them had diffusion dysfunction. Almost all patients respond well to glucocorticoid and immunosuppressant. CONCLUSIONS EGPA is rare in clinical, often involving multiple systems with great harm and may combine with asthmatic manifestations. Pulmonary involvement is relatively common. However, due to insufficient recognition of this disease and huge heterogeneity of pulmonary imaging manifestations, misdiagnosis and missed diagnosis are easy to occur. Relevant laboratory, imaging, and biopsy examination should be performed as early as possible with comprehensive consideration of extrapulmonary involvement. Early identification has great significance to improve the diagnosis rate and prognosis of diseases.
Humans ; Male ; Female ; Middle Aged ; Aged ; Churg-Strauss Syndrome/pathology* ; Granulomatosis with Polyangiitis/pathology* ; Retrospective Studies ; Lung/pathology* ; Asthma