A CASE OF PULMONARY AMYLOIDOSIS WITH PROGRESSIVE SYSTEMIC INVOLVEMENT

The authors report an unusual presentation of juvenile xanthogranuloma (JXG), a non–Langerhans cell histiocytosis of infancy and early childhood. This entity typically presents as a cutaneous head or neck nodule but can manifest with more systemic involvement including in the central nervous system. However, currently there is limited information regarding specific imaging features differentiating JXG from other neuropathological entities, with diagnosis typically made only after tissue sampling. The authors reviewed the initial images of a young patient with shunt-treated hydrocephalus and enlarging, chronic, extraaxial processes presumed to reflect subdural collections from overshunting, and they examine the operative discovery of a mass lesion that was pathologically proven to be JXG. Their results incorporate the important associated histological and advanced imaging features, including previously unreported metabolic activity on FDG PET. Ultimately, the case underscores the need to consider JXG in differential diagnoses of pediatric intracranial masses and highlights the potential role of PET in the initial diagnosis and response to treatment.

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Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review

Pathogens ◽

10.3390/pathogens10010031 ◽

2021 ◽

Vol 10 (1) ◽

pp. 31

Author(s):

Céline Betti ◽

Pietro Camozzi ◽

Viola Gennaro ◽

Mario G. Bianchetti ◽

Martin Scoglio ◽

...

Keyword(s):

Mycoplasma Pneumoniae ◽

Legionella Pneumophila ◽

Leukocytoclastic Vasculitis ◽

Systemic Vasculitis ◽

Bacterial Pathogen ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Systemic Involvement ◽

Symptomatic Disease ◽

Atypical Pathogen

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.

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POS0119 RENAL INVOLVEMENT AND NEED OF RENAL REPLACEMENT THERAPY IN ANCA ASSOCIATED VASCULITIS IN A SPANISH SINGLE-CENTER STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.345 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 270.2-271

Author(s):

J. Álvarez Troncoso ◽

J. C. Santacruz Mancheno ◽

A. Díez Vidal ◽

S. Afonso Ramos ◽

A. Noblejas Mozo ◽

...

Keyword(s):

Renal Replacement Therapy ◽

Replacement Therapy ◽

Granulomatosis With Polyangiitis ◽

Renal Involvement ◽

Age At Diagnosis ◽

Systemic Involvement ◽

Risk Of Death ◽

Anca Associated Vasculitis ◽

The Mean

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared

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Central Nervous System Vasculitis as a Rare Presentation of Mycoplasma pneumoniae: A Case Report

Case Reports in Neurology ◽

10.1159/000510632 ◽

2020 ◽

Vol 12 (3) ◽

pp. 402-409

Author(s):

Ashraf Omer Elamin Ahmed ◽

Mona Mohammad Ibraheem Babikir ◽

Amir Elssoni Mahjoup Khojali ◽

Suresh Nalaka Menik Arachchige ◽

Abdirahman Mohamud Abdirahman ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Lower Respiratory Tract ◽

Respiratory Tract Infections ◽

Central Nervous System Involvement ◽

Lower Respiratory Tract Infections ◽

Central Nervous System Vasculitis ◽

Rare Presentation ◽

Systemic Involvement ◽

Tract Infections

<i>Mycobacteria pneumoniae</i> (MP) commonly causes upper and lower respiratory tract infections. The clinical manifestation is classified as pulmonary and extrapulmonary. These manifestations vary according to the involved system. MP may affect one system or more at a time. Commonly prodromal respiratory symptoms precede systemic involvement. Central nervous system involvement in uncommon. This report is presenting a rare case of central nervous system vasculitis secondary to MP, highlighting the diagnosis and management with a succinct literature review.

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A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis

Oxford Medical Case Reports ◽

10.1093/omcr/omaa134 ◽

2021 ◽

Vol 2021 (2) ◽

Author(s):

Feihong Ding ◽

Yun Li ◽

Shailesh Balasubramanian ◽

Subha Ghosh ◽

Jason N Valent ◽

...

Keyword(s):

Light Chain ◽

Systemic Disease ◽

Amyloid Deposition ◽

Misfolded Proteins ◽

Immunoglobulin Light Chain ◽

Anatomic Site ◽

Unique Case ◽

Pulmonary Amyloidosis ◽

Immunoglobulin Light Chain Amyloidosis ◽

Tracheobronchial Amyloidosis

ABSTRACT Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition.

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Atypical and Rare Forms of Cutaneous Lupus Erythematosus: The Importance of the Diagnosis for the Best Management of Patients

Dermatology ◽

10.1159/000515766 ◽

2021 ◽

pp. 1-10

Author(s):

Astrid Herzum ◽

Giulia Gasparini ◽

Emanuele Cozzani ◽

Martina Burlando ◽

Aurora Parodi

Keyword(s):

General Practitioner ◽

Autoimmune Disease ◽

Lupus Erythematosus ◽

Cutaneous Lupus Erythematosus ◽

Cutaneous Manifestations ◽

Skin Manifestation ◽

Systemic Involvement ◽

Best Management ◽

Wide Range ◽

Cutaneous Lupus

Lupus erythematosus (LE) is an autoimmune disease with a wide range of clinical and cutaneous manifestations. Along with the well-known typical cutaneous manifestations of LE, some cutaneous manifestations are rarer, but still characteristic, enabling the dermatologist and the general practitioner who know them to suspect cutaneous LE (CLE) and investigate a possible underlying systemic involvement. Indeed, not infrequently a skin manifestation is the first presentation of systemic LE (SLE), and >75% of SLE patients show signs of skin disease during the course of the illness. Especially, SLE involvement occurs in cases of acute CLE, while it is uncommon in subacute CLE and rare in chronic CLE. This review aims to concentrate especially on atypical cutaneous manifestations of LE to enable the clinician to diagnose even the rarest forms of CLE.

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Pulmonary amyloidosis and cystic lung disease in primary Sjögren’s syndrome: a case report and literature review

Clinical Rheumatology ◽

10.1007/s10067-021-05596-9 ◽

2021 ◽

Author(s):

Haiyun Li ◽

Yuewu Lu

Keyword(s):

Case Report ◽

Literature Review ◽

Lung Disease ◽

Sjogren's Syndrome ◽

Primary Sjögren’S Syndrome ◽

Primary Sjogren’S Syndrome ◽

Primary Sjögren's Syndrome ◽

Cystic Lung ◽

Cystic Lung Disease ◽

Pulmonary Amyloidosis

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A Case of Atypical Bartonellosis in a 4-Year-Old Immunocompetent Child

Microorganisms ◽

10.3390/microorganisms9050950 ◽

2021 ◽

Vol 9 (5) ◽

pp. 950

Author(s):

Chiara Sodini ◽

Elena Mariotti Zani ◽

Francesco Pecora ◽

Cristiano Conte ◽

Viviana Dora Patianna ◽

...

Keyword(s):

Clinical Laboratory ◽

Bartonella Henselae ◽

Delayed Diagnosis ◽

Mitral Valve Regurgitation ◽

Low Grade ◽

Systemic Involvement ◽

Mild Disease ◽

Precise Diagnosis ◽

Major Illness ◽

The Right

In most cases, infection due to Bartonella henselae causes a mild disease presenting with a regional lymphadenopathy frequently associated with a low-grade fever, headache, poor appetite and exhaustion that spontaneously resolves itself in a few weeks. As the infection is generally transmitted by cats through scratching or biting, the disease is named cat scratch disease (CSD). However, in 5–20% of cases, mainly in immunocompromised patients, systemic involvement can occur and CSD may result in major illness. This report describes a case of systemic CSD diagnosed in an immunocompetent 4-year-old child that can be used as an example of the problems that pediatricians must solve to reach a diagnosis of atypical CSD. Despite the child’s lack of history suggesting any contact with cats and the absence of regional lymphadenopathy, the presence of a high fever, deterioration of their general condition, increased inflammatory biomarkers, hepatosplenic lesions (i.e., multiple abscesses), pericardial effusion with mild mitral valve regurgitation and a mild dilatation of the proximal and medial portion of the right coronary artery, seroconversion for B. henselae (IgG 1:256) supported the diagnosis of atypical CSD. Administration of oral azithromycin was initiated (10 mg/kg/die for 3 days) with a progressive normalization of clinical, laboratory and US hepatosplenic and cardiac findings. This case shows that the diagnosis of atypical CSD is challenging. The nonspecific, composite and variable clinical features of this disease require a careful evaluation in order to achieve a precise diagnosis and to avoid both a delayed diagnosis and therapy with a risk of negative evolution.

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Flunixin Meglumine Reduces Milk Isoprostane Concentrations in Holstein Dairy Cattle Suffering from Acute Coliform Mastitis

Antioxidants ◽

10.3390/antiox10060834 ◽

2021 ◽

Vol 10 (6) ◽

pp. 834

Author(s):

Carsten C. F. Walker ◽

Jill L. Brester ◽

Lorraine M. Sordillo

Keyword(s):

Medical Intervention ◽

Systemic Involvement ◽

Analgesic Effects ◽

Markers Of Inflammation ◽

Inflammatory Mediator Production ◽

Flunixin Meglumine ◽

Coliform Mastitis ◽

Cox 2 ◽

Anti Inflammatory ◽

Oxidant Status

Dysfunctional inflammation contributes significantly to the pathogenesis of coliform mastitis and the classical pro-inflammatory enzyme cyclooxygenase-2 (COX-2) is the target of medical intervention using the non-steroidal anti-inflammatory drug (NSAID) flunixin meglumine (FM). Inhibition of COX-2 by FM can decrease concentrations of pro-inflammatory fatty acid-based mediators called eicosanoids, providing antipyretic and analgesic effects in dairy cows suffering from coliform mastitis. However, approximately 50% of naturally occurring coliform mastitis with systemic involvement results in death of the animal, even with NSAID treatment. Inadequate antioxidant potential (AOP) to neutralize reactive oxygen species (ROS) produced during excessive inflammation allows for oxidative stress (OS), contributing to tissue damage during coliform mastitis. Biomarkers of lipid peroxidation by ROS, called isoprostanes (IsoP), were used in humans and cattle to quantify the extent of OS. Blood IsoP were shown to be elevated and correlate with oxidant status during acute coliform mastitis. However, the effect of FM treatment on oxidant status and markers of OS has not been established. Blood IsoP concentrations were used to quantify systemic OS, whereas milk was used to assess local OS in the mammary gland. Results indicate that FM treatment had no effect on blood markers of inflammation but reduced the oxidant status index (OSi) by increasing blood AOP from pre- to post-FM treatment. Milk AOP significantly increased from pre- to post-FM treatment, whereas ROS decreased, resulting in a decreased OSi from pre- to post-FM treatment. The only blood IsoP concentration that was significantly different was 5-iso-iPF2α-VI, with a decreased concentration from pre- to post-FM treatment. Conversely, milk 5-iso-iPF2α-VI, 8,12-iso-iPF2α-VI, and total IsoP concentrations were decreased following FM treatment. These results indicated that administration of FM did improve systemic and local oxidant status and reduced local markers of OS. However, differential effects were observed between those animals that survived the infection and those that died, indicating that pre-existing inflammation and oxidant status greatly affect efficacy of FM and may be the key to reducing severity and mortality associated with acute coliform infections. Supplementation to improve AOP and anti-inflammatory mediator production may significantly improve efficacy of FM treatment.

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