scholarly journals Granulomatosis with Polyangiitis Presenting as Pyrexia of Unknown Origin, Leukocytosis, and Microangiopathic Haemolytic Anemia

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Sima Terebelo ◽  
Iona Chen
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Kidney Injury ◽  
Unknown Origin ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
High Dose ◽  
Normal Range ◽  
Pyrexia Of Unknown Origin ◽  
History Of ◽  
Haemolytic Anemia

A 66-year-old woman presented to the Emergency Department with a florid sepsis-like picture, a two-week history of fever, relative hypotension with end organ ischemia (unexplained liver enzyme and troponin elevations), and nonspecific constitutional symptoms. She was initially found to have a urinary tract infection but, despite appropriate treatment, her fever persisted and her white blood cell count continued to rise. During her hospitalization the patient manifested leukocytosis to 47,000 WBC/μL, ESR 67 mm/hr (normal range 0–42 mm/hr), CRP 17.5 mg/dL (normal range 0.02–1.20 mg/dL), and microangiopathic haemolytic anemia, with declining haemoglobin and haematocrit. An infectious aetiology was not found despite extensive bacteriologic studies and radiographic imaging. The patient progressed to acute kidney injury with “active” urinary sediment and proteinuria. Kidney biopsy results and serological titres of myeloperoxidase positive perinuclear-antineutrophil cytoplasmic antibodies (MPO+ p-ANCA) led to a diagnosis of granulomatosis with polyangiitis. Immunosuppressive treatment with high dose methylprednisolone and rituximab led to resolution of the leukocytosis and return of the haemoglobin and haematocrit values toward normal without further signs of hemolysis.

scholarly journals Rosuvastatin-related rhabdomyolysis causing severe proximal paraparesis and acute kidney injury

2019 ◽  
Vol 12 (10) ◽  
pp. e229244 ◽  
Author(s):  
Kosar Hussain ◽  
Anil Xavier
Keyword(s):  
Acute Kidney Injury ◽  
Kidney Injury ◽  
High Dose ◽  
Normal Range ◽  
Limb Weakness ◽  
Lower Limb Weakness ◽  
History Of ◽  
Complicated Course ◽  
Bilateral Lower Limb ◽  
Initial Blood

We describe the case of a 76-year-old man who presented with bilateral lower limb weakness associated with decreased urine output. His initial blood results showed acute kidney injury (AKI) stage 3 with substantially raised serum creatine kinase concentration of 37 950 IU/L (normal range <171 U/L). He had been on high-dose rosuvastatin for 4 years with a recent brand change occurring 1 week prior to onset of symptoms. There was no history of pre-existing neuromuscular disease. Statin-related rhabdomyolysis was suspected and rosuvastatin was withheld. His muscle strength gradually improved. He required haemodialysis for 10 weeks. He was discharged home after a complicated course of hospitalisation. His renal function improved and he became dialysis-independent; however, he was left with residual chronic kidney disease.

scholarly journals Eosinophilic granulomatosis with polyangiitis in a Nigerian woman

2019 ◽  
Vol 12 (6) ◽  
pp. e228901
Author(s):  
Ngozi Lina Ekeigwe ◽  
Olufemi Adelowo ◽  
Ehiaghe Lonia Anaba ◽  
Hakeem Olaosebikan
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rare Condition ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Systemic Autoimmune Disease ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Nigerian Woman ◽  
History Of ◽  
Perinuclear Anca ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. There were also associated symptoms of peripheral neuropathy, weight loss, peripheral blood eosinophilia and positive perinuclear ANCA and she was previously managed for rheumatoid arthritis and asthma. She fulfilled the American College of Rheumatology criteria for EGPA and was treated with glucocorticoids and methotrexate which led to significant improvement of her symptoms.

scholarly journals EP05 The wolf finds unusual prey

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Mithun Chakravorty ◽  
Robert Sandler ◽  
Ayman El-Nayal
Keyword(s):  
Lupus Erythematosus ◽  
Late Onset ◽  
Obstructive Uropathy ◽  
Kidney Injury ◽  
Unknown Origin ◽  
Renal Calculus ◽  
Tertiary Hospital ◽  
Haemodynamic Instability ◽  
Pyrexia Of Unknown Origin ◽  
Critical Care Admission

Abstract Background Systemic lupus erythematosus (SLE) or lupus (Latin for wolf) is a rare multi-system autoimmune disease that has usually presented by the fifth decade, with a seven-fold higher incidence in females, especially of Afro-Caribbean descent. It is a recognised cause of pyrexia of unknown origin (PUO) though infection and malignancy are far commoner causes. Lupus nephritis (LN) can be life-threatening and affects nearly one-third of patients. We highlight a surprising case of late-onset SLE in a 65-year-old Caucasian man that presented with PUO and early LN. Methods Our patient was known to have atrial fibrillation, hypertension and previous obstructing renal calculus and presented to the Emergency Department with two weeks of right flank pain and fevers. He required critical care admission due to haemodynamic instability. CT excluded obstructive uropathy but a complex right renal cyst was present with mild inflammatory stranding. Bloods showed new neutrophilia (8.31-9.50 x 109/L), anaemia (haemoglobin 93 g/L), thrombocytopenia (platelets 90 x 109/L) and acute kidney injury (creatinine 128 μmol/l from 90 μmol/l baseline). CRP persisted above 110 mg/L. Liver function and ferritin were normal. Despite escalation of antibiotics he remained febrile, creatinine peaked at 291 μmol/L with raised urine protein:creatinine ratio (PCR) of 384 mg/mmol. Repeated septic screens and blood-borne virus tests were negative. Further CT showed mild ascites and pleural effusions. Transthoracic echocardiogram revealed a small pericardial effusion only, and pleural aspirate was a transudate, without malignant cells. Results There was clinical improvement after three weeks despite persistent fevers and he was discharged. However, readmission within three days necessitated inotropic support. After stabilisation, he was transferred under Infectious Diseases in a tertiary hospital. Shortly afterwards, the autoimmune screen showed positive anti-nuclear antibody (ANA), with positive Crithidia luciliae IgG double-stranded DNA (dsDNA) and titres &gt;379 IU/mL. Concurrent hypocomplementaemia (C3 0.22 g/L, C4 0.03 g/L) raised the possibility of LN with SLE and renal biopsy demonstrated class 3 LN. Prompt treatment with intravenous glucocorticoids and mycophenylate mofetil (MMF) resulted in an excellent recovery. Almost a year later, he is taking MMF 1g twice daily with prednisolone 10mg daily. His creatinine is stable at 110 μmol/L, urine PCR around 300 mg/mmol and complements have normalised, despite persistent dsDNA titre of 150 IU/mL. He continues driving taxis and reports a good quality of life. Conclusion There was low suspicion of SLE in this case given the absence of typical symptoms and considering his age, gender and ethnicity. A literature review of male patients with SLE suggested tendency for later-onset and a worse prognosis. There appears to be less LN but when present requires prompt immunosuppression as occurred in this case. Currently, the oldest documented male patient with both SLE and biopsy-proven LN at diagnosis was 74 years old. Disclosures M. Chakravorty None. R. Sandler None. A. El-Nayal None.

scholarly journals The wrong Spirochaete? Acute kidney injury in a returning traveller with syphilis – a case report

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Hannah Cook ◽  
Mark Gompels
Keyword(s):  
Acute Kidney Injury ◽  
Case Report ◽  
Acute Infection ◽  
Kidney Injury ◽  
Unknown Origin ◽  
Improve Patient Care ◽  
Risk Groups ◽  
Sexual History ◽  
Pyrexia Of Unknown Origin ◽  
Sex With Men

Abstract Background Syphilis has seen an increased incidence in recent years and can have serious and irreversible consequences if left un-diagnosed and untreated. This case report describes a presentation of syphilis and acute kidney injury – a scenario sparsely described in existing literature. Case presentation This 43-year old Man who has Sex with Men (MSM) presented to the emergency department with a 3-week history of vomiting and headaches, progressing to include pyrexia. These symptoms started following his return from a 2-week cruise in Central America throughout which he had been well. He had a background of well-controlled human immunodeficiency virus (HIV). On admission he had an Acute Kidney Injury (AKI) stage 3, without hydronephrosis, presumed to be pre-renal. Leptospirosis, the main differential, was negative serologically. ‘Pyrexia of unknown origin’ testing was performed, and cefuroxime commenced. Later in the admission, syphilis testing indicated an acute infection and he completed a full treatment course of benzylpenicillin. This, alongside intravenous fluids, resulted in symptom and renal resolution in 9 days and restoration of renal function. Conclusions Renal complications in syphilis are rare, furthermore the majority of those documented occur in latent syphilis and are irreversible. There are limited numbers of other documented cases of AKI in acute syphilis, which like the gentleman in this case were reversible and did not lead to permanent kidney damage. This case adds to the knowledge base of AKI in initial presentation of syphilis. It also demonstrates not only the importance of taking a sexual history in patients with new infective symptoms but that testing for syphilis in at-risk groups regardless of history should be performed given its rising incidence. These considerations by physicians can lead to prompt diagnosis and management of syphilis and improve patient care and long-term outcomes.

scholarly journals A case of eosinophilic granulomatosis with polyangiitis after prolonged intervals of an anti-interleukin-6 receptor antibody for rheumatoid arthritis

2021 ◽  
Author(s):  
Yuki Imai ◽  
Yasushi Kondo ◽  
Sho Ishigaki ◽  
Naoshi Nishina ◽  
Yuichiro Ota ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Granulomatosis With Polyangiitis ◽  
Allergic Diseases ◽  
Pericardial Fluid ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Upper Gastrointestinal Endoscopy ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Receptor Antibody ◽  
Eosinophilic Granulomatosis

ABSTRACT An 83-year-old woman with a history of asthma complained of left abdominal pain and was admitted to our hospital. She was treated with tocilizumab, an anti-interleukin (IL)-6 receptor antibody, with a prolonged interval for rheumatoid arthritis (RA). Laboratory tests revealed a remarkable increase in eosinophil count and inflammatory markers with negative antineutrophil cytoplasmic antibodies. Echocardiography revealed pericardial fluid retention, and contrast-enhanced computed tomography revealed the thickening of the gastric antrum wall. Upper gastrointestinal endoscopy and biopsy revealed eosinophilic infiltration into the gastric mucosal epithelium. She was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) with pericarditis and eosinophilic gastroenteritis. High-dose glucocorticoids with intermittent intravenous cyclophosphamide (IVCY) were initiated, resulting in remission. As IL-6 is involved in the pathogenesis of allergic diseases such as asthma, our case can provide insights into the pathogenic role of IL-6 in EGPA as the development of EGPA in our case may have been triggered by IL-6 signals enhanced with tocilizumab interval prolongation.

scholarly journals New Treatment Options for Pituitary Granulomatosis With Polyangiitis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A594-A594
Author(s):  
Hessa Boharoon ◽  
Majid AlAmeri ◽  
Abdulla Mohamed Alnuaimi ◽  
Nigel Mendoza ◽  
Stephen McAdoo ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Treatment Guidelines ◽  
Dynamic Assessment ◽  
Treatment Options ◽  
Case Series ◽  
Rare Condition ◽  
High Dose ◽  
Pituitary Mass ◽  
Remission Maintenance ◽  
History Of

Abstract Background: Granulomatosis with polyangiitis (GPA) rarely involves the pituitary gland and has been reported in 1% of all cases of GPA. Most frequently, it presents with pituitary mass effect and diabetes insipidus (DI). To date, there are no treatment guidelines for this rare condition. Case Presentation: Case 1: A 55 year old female with a history of ANCA-positive pulmonary GPA, previously treated with glucocorticoids and immunosuppressants, presented two years later with cranial DI and bitemporal hemianopia. MRI showed a large sellar mass with suprasellar extension. High dose glucocorticoids resulted in good clinical and radiological response. Further treatment consisted in a combination of Cyclophosphamide and Rituximab (RTX). Further doses of RTX are planned aiming for a period of B-cell depletion. Case 2: A 38 year old female, presented with polyuria, recurrent nosebleeds, headaches and a left visual field defect. Pituitary profile revealed ACTH deficiency and MRI showed a heterogenous cystic lesion with peripheral enhancement and stalk thickening. Steroid replacement led to immediate improvement in her symptoms. Cranial DI was confirmed and raised Proteinase 3 (PR3) antibody suggested GPA. A combination of prednisolone and Methotrexate led to significant improvement of MRI appearances and declining PR3 antibody levels. For remission maintenance, two cycles of RTX were given with further radiological and biochemical improvement, and, following dynamic assessment of her HPA axis, she could be fully weaned off steroids. Case 3 is a 47 year old female with a history of childhood asthma. She was found to have cavitating lung lesions. ANCA positivity confirmed GPA and she was commenced on high-dose steroids. During follow-up, she developed headache, polyuria and polydipsia. MRI pituitary showed a suprasellar lesion and pituitary biopsy revealed inflammatory hypophysitis. Cranial DI was confirmed by water deprivation testing. Previous allergic reactions to both RTX and Ofatumumab precluded anti-CD40 monoclonal antibodies and she was commenced on Azathioprine. A further recurrence of pituitary GPA necessitated escalation of the steroid dose and switch of azathioprine to mycophenolate mofetil. She remains in remission and her steroids reduced to a maintenance dose. Conclusion: GPA pituitary has been observed to occur at variable time after diagnosis often in the absence of any other systemic features. A combination of glucocorticoids and RTX has been approved for severe relapsing pulmonary GPA, however, limited data is available for pituitary GPA. In this case series, the response to high dose steroids and RTX for remission maintenance has been encouraging. Experience with ‘conventional’ immunosuppresants remains limited and therapeutic responses remain variable. Further clinical studies are required to establish effective treatment for pituitary GPA.

La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore

2020 ◽  
Vol 39 (9) ◽  
pp. 569-574
Author(s):  
Matteo Pavan ◽  
Anna Agrusti ◽  
Andrea Trombetta ◽  
Serena Pastore ◽  
Alberto Tommasini ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Inflammation ◽  
Peripheral Eosinophilia ◽  
Churg Strauss Syndrome ◽  
History Of ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

scholarly journals PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Athira Unnikrishnan ◽  
Shila Azodi ◽  
Nadeem Ansari ◽  
Megan Brown ◽  
Joshua Kamnetz ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Granulomatosis With Polyangiitis ◽  
Brain Biopsy ◽  
High Dose ◽  
Review Of Literature ◽  
Left Hemiparesis ◽  
Cerebral Angiogram ◽  
History Of ◽  
Unilateral Proptosis ◽  
The Brain

PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis (UC) patients, the clinical implication of which is not well defined. We are reporting a case of 27-year-old Caucasian male with 14-year history of UC presenting with unilateral proptosis, conjunctival congestion, and chemosis who developed acute hemiparesis within three days of hospital admission, followed by rapid neurological deterioration correlating with brain imaging findings. Serologically he had atypical PANCA with high PR3 antibody titer with a negative infectious workup. His cerebral angiogram was normal but the brain biopsy showed necrotizing vasculitis. He was diagnosed with PR3 ANCA mediated cerebral and orbital vasculitis associated with UC. Treatment was initiated with high dose steroids, plasmapheresis, and cyclophosphamide. He improved significantly with residual left hemiparesis.

scholarly journals 330. Risk Factors for Acute Kidney Injury after the Use of Antibiotic Loaded Bone Cement in Orthopedic Surgery – a Retrospective Case-control Study

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S237-S237
Author(s):  
Lucas Schulz ◽  
Darina Georgieva ◽  
Ambar Haleem
Keyword(s):  
Acute Kidney Injury ◽  
Bone Cement ◽  
Orthopedic Surgery ◽  
Elevated Serum ◽  
Kidney Injury ◽  
Case Control ◽  
High Dose ◽  
Cement Spacer ◽  
Close Monitoring ◽  
History Of

Abstract Background As the number of joint replacement surgeries continues to rise, so does the number of joint infections. Many patients end up needing the implantation of antibiotic loaded bone cement (ALBC) to treat their infection. The use of localized high dose vancomycin, tobramycin, and gentamicin may be linked to acute kidney injury (AKI) in certain patients. Our hypothesis is that patients who developed AKI after receiving a joint spacer had a predisposition to AKI due to other comorbidities, high antibiotic doses in ALBC, immunosuppression, or the use of other nephrotoxic drugs pre-op. These patients may need close monitoring of their renal function and serum antibiotic levels after surgery. Methods We performed a chart review of 428 patients who underwent an orthopedic surgery that involved insertion of ALBC at our institution between 2015 and 2018. We excluded patients under age 18, those who had antibiotic irrigation only, trauma patients, non-arthroplasty surgeries (such as fractures and debridement of deep wounds), and patients with missing data for 30 days after the surgery. We identified 57 patients who fit our inclusion criteria and received a bone cement spacer or beads to treat an infection of the hip, knee, shoulder, or ankle. We matched patients who had AKI to 2 patients who did not have AKI. Matching was based on age (± 5 years), joint operated on, and antibiotics used. Results 15 patients showed an elevated serum creatinine level of over 1.2 within 30 days of surgery. 86.7% of these patients were male, their average age was 64.1 ± 6.2 years old, 40% had hip surgery, 46.7% knee surgery, 6.7% ankle, and 6.7% shoulder. All received vancomycin and tobramycin in Palacos bone cement. Compared to their case-control matches, these patients had more frequent use of immunosuppressive medication, a history of malignancy, a history of previous kidney disease, and obesity. The use of combined intravenous vancomycin and piperacillin-tazobactam post-operatively may also be linked to higher rates of AKI. Conclusion Immunosuppression, obesity, male gender, and history of kidney injury and cancer are factors associated with AKI after ALBC spacer implantation. Further analysis and study are needed to identify potential causation between ALBC use and AKI. Disclosures All Authors: No reported disclosures

scholarly journals Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis

Reumatismo ◽  
2019 ◽  
Vol 71 (2) ◽  
pp. 92-98
Author(s):  
D.J. Manzella ◽  
L. Vicente ◽  
A.A. Pérez de la Hoz ◽  
R.J. Zamora ◽  
G. De Rosa ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Antiphospholipid Syndrome ◽  
Clinical Data ◽  
Antinuclear Antibodies ◽  
Elevated Serum ◽  
Kidney Injury ◽  
Multiple Organ ◽  
Normal Range ◽  
Catastrophic Antiphospholipid Syndrome ◽  
History Of

A 62-year-old man with a history of systemic sclerosis was admitted with diffuse alveolar hemorrhage and acute kidney injury without clinical data suggestive of glomerulonephritis. Laboratory tests showed anemia, leukocytosis with neutrophilia, thrombocytopenia, elevated serum creatinine and metabolic acidosis. Antinuclear antibodies were positive at a titer of 1/640 (speckled, 1/160; nucleolar, 1/320) while rheumatoid factor, anti Scl-70, anti-centromere, anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibodies were negative and serum complement levels were within normal range. During the following days, the patient developed multiple organ failure and, eventually, died. Lupus anticoagulant was revealed positive after the patient’s death, suggesting a catastrophic antiphospholipid syndrome. Clinical data and autopsy were consistent with this diagnosis.

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