Severe Systemic Vasculitis Research Articles

Serum sulfatide level is associated with severe systemic vasculitis with kidney involvement.

Sulfatides are a type of sulfated glycosphingolipid that are secreted with lipoproteins into the serum. These molecules are involved in the inflammatory pathway of vessels in addition to coagulation and platelet aggregation. Previous studies have proposed that sulfatides play a pivotal role in regulating inflammation-related disorders. Systemic vasculitis (SV) diseases are generally caused by autoimmune diseases and often involve kidney vasculitis, which may lead to rapidly progressive kidney dysfunction and end-stage kidney disease. Our earlier pilot study revealed that the level of serum sulfatides (SSs) was significantly decreased in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), a representative disease-causing SV with kidney involvement (SVKI), especially in patients exhibiting active crescentic findings on kidney biopsy. To further explore the clinical significance of an association between SS and SVKI, we analyzed and compared the SS level of patients with various SVKI diseases in this retrospective cohort study. Among patients admitted to our hospital between 2008 and 2021, we ultimately enrolled 26 patients with IgA vasculitis (IgAV), 62 patients with AAV, and 10 patients with anti-glomerular basement membrane disease (GBM) as examples of SVKI diseases, as well as 50 patients with IgA nephropathy (IgAN) and 23 donors for living kidney transplantation as controls. The mean ± standard deviation SS level in the donor, IgAN, IgAV, AAV, and GBM groups was 8.26 ± 1.72, 8.01 ± 2.21, 6.01 ± 1.73, 5.37 ± 1.97, and 2.73 ± 0.99 nmol/mL, respectively. Analysis of patients in the SVKI disease group showed that those with the crescentic class kidney biopsy finding exhibited a significantly lower SS level than did those with other class biopsy features. Additionally, the SS level had a higher detection ability for SVKI patients with crescentic class kidney biopsy findings (area under the receiver operating characteristic curve 0.90, 95% confidence interval 0.82-0.99) than did several other predictor candidates. Our results indicate that the SS level is decreased in more severe SVKI diseases and may be associated with active glomerular lesions in SVKI kidney biopsy samples.

Vascularite systémique sévère induite par l’ibrutinib

IntroductionThe specific cutaneous toxicity of Bruton's tyrosine kinase inhibitors is poorly described. We report a case of severe systemic vasculitis induced by ibrutinib. ObservationA 73-year-old woman with chronic lymphocytic leukemia was treated with ibrutinib. Eighteen months after treatment onset, ulceronecrotic lesions on toes and tongue occurred. Skin biopsy found vasculitis of small and medium vessels. Biologic tests were negative. This vasculitis was refractory to systemic corticosteroid therapy and azathioprine. Ibrutinib was stopped on the hypothesis of drug-induced vasculitis. Skin lesions improved after discontinuation of ibrutinib. ConclusionThe mechanism of action of ibrutinib does not explain the occurrence of vasculitis and an immunoallergic mechanism is suspected.

Combined central retinal artery occlusion and anterior ischemic optic neuropathy as presenting signs of systemic lupus erythematosus

Systemic Lupus Erythematosus (SLE) is a chronic auto-immune condition with systemic and ocular involvement. Ophthalmological findings are diverse and can involve all layers of the globe. Posterior segment manifestations can include lupus retinopathy, retinal vascular occlusions and lupus choroidopathy. We report here a rare case of central retinal artery occlusion (CRAO) associated with anterior ischemic optic neuropathy (AION) in a young female patient as presenting signs of SLE. In this case report, we highlight a very uncommon initial presentation of this severe systemic vasculitis. A multidisciplinary approach is required in order to prevent life-threatening vaso-occlusive complications.

Differential diagnosis of a local form of granulomatosis with polyangiitis: nasal cavity and paranasal sinuses lesions (part 1)

Granulomatosis with polyangiitis (GPA) is a primary vasculitis associated with antineutrophil cytoplasmic antibodies, characterized by necrotizing vasculitis with predominant involvement of small vessels of various localizations and necrotizing granulomatous inflammation with multiple clinical manifestations. GPA remains one of the most severe systemic vasculitis with unfavorable prognosis. When analyzing the course of the disease, there are two variants of GPA, local (with lesions of the upper respiratory tract, URT, organs of vision and hearing) and generalized (with lesions of the URT, organs of vision and hearing in combination with the lungs and/or kidneys, gastrointestinal tract, nervous systems, skin involvement).The article discusses the differential diagnosis of the disease with the nasal cavity and paranasal sinuses lesions onset, which requires an interdisciplinary approach and interaction of doctors of different specialties.

Patient outcome in antibody-positive systemic vasculitis treated with therapeutic plasma exchange.

Therapeutic plasma exchange (TPE) has been advocated as an adjunct to steroids and cytotoxic drugs in treating patients suffering from vasculitis and presenting with active disease, but we still have insufficient evidence on its effectiveness in improving the clinical response, especially in India. This study was planned to study the clinical outcome in severe vasculitic presentations treated with TPE as an adjunctive therapy. A retrospective analysis of TPE procedures performed from July 2013 to July 2017 in the department of transfusion medicine at a large tertiary care hospital was done. All consecutive patients admitted with new diagnosis of systemic vasculitis presenting with active disease and severe presentations such as advanced renal failure or severe respiratory abnormalities or life-threatening vasculitis affecting the gastrointestinal tract, neurological and musculoskeletal system; who needed TPE for removal of preformed antibodies, were included in the study. There were a total of 31 patients in whom TPE was performed for severe systemic vasculitis; 26 adults and five pediatric. Six patients tested positive for perinuclear fluorescence, 13 for cytoplasmic fluorescence (cANCA), two for atypical antineutrophil cytoplasmic autoantibody, seven for anti-glomerular basement membrane antibodies, two for antinuclear antibodies (ANA), and one patient tested positive for ANA as well as cANCA before the augmentation of TPE. Out of 31, seven patients showed no clinical improvement and succumbed to the disease. At the end of desired number of procedures, 19 tested negative and five tested weak positive for their respective antibodies. Favorable clinical outcomes were observed with TPE in patients with antibody-positive systemic vasculitis.

Pathobiology of highly pathogenic H5 avian influenza viruses in naturally infected Galliformes and Anseriformes in France during winter 2015\u20132016

In late 2015, an epizootic of Highly Pathogenic Avian Influenza (H5Nx) was registered in Southwestern France, including more than 70 outbreaks in commercial poultry flocks. Phylogenetic analyses suggested local emergence of H5 viruses which differed from A/goose/Guangdong/1/1996 clade 2.3.4.4b lineage and shared a unique polybasic cleavage site in their hemagglutinin protein. The present work provides an overview of the pathobiological picture associated with this epizootic in naturally infected chickens, guinea fowls and ducks. Upon necropsy examination, selected tissues were sampled for histopathology, immunohistochemistry and quantitative Real Time Polymerase Chain Reaction. In Galliformes, HPAIVs infection manifested as severe acute systemic vasculitis and parenchymal necrosis and was associated with endothelial expression of viral antigen. In ducks, lesions were mild and infrequent, with sparse antigenic detection in respiratory and digestive mucosae and leukocytes. Tissue quantifications of viral antigen and RNA were higher in chickens and guinea fowls compared to duck. Subsequently, recombinant HA (rHA) was generated from a H5 HPAIV isolated from an infected duck to investigate its glycan-binding affinity for avian mucosae. Glycan-binding analysis revealed strong affinity of rHA for 3’Sialyl-LacNAc and low affinity for Sialyl-LewisX, consistent with a duck-adapted virus similar to A/Duck/Mongolia/54/2001 (H5N2). K222R and S227R mutations on rHA sequence shifted affinity towards Sialyl-LewisX and led to an increased affinity for chicken mucosa, confirming the involvement of these two mutations in the glycan-binding specificity of the HA. Interestingly, the rHA glycan binding pattern of guinea fowl appeared intermediate between duck and chicken. The present study presents a unique pathobiological description of the H5 HPAIVs outbreaks that occurred in 2015–2016 in Southwestern France.

A case report of a severe neonatal systemic vasculitis on the first day of life

BackgroundNeonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’s syndrome, Behҫet’s disease, cutaneous polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitides. Here we report a novel association of a case of new-onset maternal seronegative inflammatory arthritis associated with a transient systemic vasculitis in a neonate.Case presentationIn the first 24 h of life, a preterm Caucasian baby boy was noted to have blue discoloration to all four extremities. Despite antibiotics, fresh frozen plasma and anticoagulation, the discoloration remained, particularly in the left index finger. This was associated with fever and a maximum C-reactive protein (CRP) of 148 mg/L. Intravenous immunoglobulin (IVIG) was given with short-term improvement. Initial echocardiogram showed enlarged coronary arteries with normalization on repeat 1 week later. Clinical signs and symptoms responded to high dose oral steroid administration. MRI angiography (MRA) of the body and heart showed tortuosity of arteries in the upper and lower extremities with gadolinium uptake, suggestive of vasculitis. Autoantibody profile negative. Genetic panel for hereditary autoinflammatory diseases was negative as was whole exome sequencing performed on the trio. The baby was weaned off steroids by 5 months of age. A small distal autoamputation of the left index finger occurred.He was born to a 28-year-old woman who developed new onset severe symmetrical polyarthritis at 8 weeks gestation. This was presumed a reactive arthritis secondary to a dental infection. Infectious work up and autoantibodies were negative. She was treated with high dose prednisone for the remainder of her pregnancy.The mother was weaned off prednisone, treated with hydroxychloroquine for 8 months post-partum and remains in remission. A repeat MRA done at 1 year old showed mild residual tortuosities of the arteries in the forearms. The remainder of the medium and large vessels were within normal limits with no gadolinium enhancement to suggest active disease. The child is now 4 years old with normal growth and development.ConclusionThis is a unique case of new-onset seronegative presumed reactive arthritis in a mother with the rare development of a successfully treated medium vessel vasculitis in an infant.

Eosinophilic granulomatosis with polyangiitis: Cutaneous clinical and histopathologic differential diagnosis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, but severe systemic vasculitis that can affect skin and other organ systems. Diagnostic criteria have evolved, and many attempts have been made to classify the vasculitides based on clinical and/or histopathologic features, with an aim to develop standardized criteria. According to the EGPA Consensus Task Force recommendations, EGPA is a syndrome of asthma, eosinophilia, pulmonary infiltrates, and extrapulmonary vasculitis (such as cutaneous involvement with purpura). Histopathologic evidence of vasculitis in EGPA may be associated with eosinophilic infiltration and/or perivascular granulomatous inflammation. We review clinicopathologic criteria of this enigmatic vasculopathy.

Incidence and clinical predictors of infections in patients treated with severe systemic ANCA-associated vasculitis.

Immunosuppressive therapy has improved the outcome of ANCA-associated vasculitis (AAV), but infectious morbidity and mortality remained high. Recognizing its risk factors seems crucial for prevention, aiming to increase survival of these patients. We investigated the incidence and types of infections and assessed predictive factors in 132 patients with severe systemic AAV. Patients with lower than median incidence of total infections/patient-year during induction had lower baseline serum creatinine, dialysis requirement and Charlson comorbidity index (CCI), compared to those with higher than median incidence (P = 0.037; P = 0.024; P = 0.001; respectively). In subgroups with below and above than median number of severe infections/patient-year during induction, differences were found in baseline creatinine (P = 0.002) and dialysis requirement (P = 0.001); comparing the same cohorts during maintenance immunosuppression, baseline dialysis requirement, diabetes, CCI, and dose of cyclophosphamide (CYC) administered as induction therapy differed significantly (P = 0.019; P = 0.015; P = 0.001; P = 0.015, respectively). Severe infections were predicted by baseline serum creatinine (OR 1.002 [CI 1.001-1.003]) and pulmonary manifestation (OR 2.153 [CI 1.017-4.560]) during induction immunosuppression. In multivariable Cox regression model all-cause mortality was independently predicted by severe infection (HR 1.998 [CI 1.214-3.287]). Among the 168 positive cultures Gram-negative bacteria were responsible for blood stream infections in 33%, and respiratory tract infections in 72%. Advanced renal failure, pulmonary involvement and high degree of comorbidities increase the risk of infection in AAV. Those who suffer infection during induction immunosuppression have worse long-term survival. Our findings indicate the need for high vigilance for infections and close follow-up of comorbidities when treating AAV.

Atypical presentation of eosinophilic granulomatosis - typical presentation in an ageing population?

Severe systemic vasculitis presenting in an 80 year old female involving a presentation with recurrent pyrexia, eosinophilia, mononeuritis multiplex against a background of late onset asthma, consistent with eosinophilic vasculitis with angiitis is described. Despite appropriate management with resolution of systemic symptoms, she failed to return to baseline functionality. Systemic vasculitis is rare in this population cohort, but it is important to consider as it is treatable albeit with a guarded outlook for full neurological recovery.

Antineutrophil Cytoplasmic Antibodies and Organ-Specific Manifestations in Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a rare and often severe systemic vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). EGPA can affect multiple organ systems, but the relationships between ANCA status and the organ-specific manifestations of EGPA in previous reports were inconsistent. To investigate the association of the ANCA status with organ-specific manifestations in EGPA. We performed a systematic review of studies published before March 16, 2020, in the PubMed, Embase, Web of Science, and Cochrane Library databases. The primary outcome was the association of ANCA status with organ-specific involvements of EGPA. Odds ratios (ORs) and 95% CIs were calculated using a random-effects model. A total of 24 cross-sectional studies with 2527 patients with EGPA, including 921 ANCA-positive patients and 1606 ANCA-negative patients, were included in the meta-analysis. The significant results of pooled analyses revealed that compared with patients with EGPA with negative ANCA status, patients with EGPA with positive ANCA status had higher risks of peripheral neuropathy (OR, 1.701), renal involvement (OR, 5.097), and cutaneous purpura (OR, 1.746) and lower risks of pulmonary infiltrates (OR, 0.589) and cardiac involvement (OR, 0.427). The pooled analysis also revealed no significant association of ANCA status with asthma and involvements of the central nervous system, gastrointestinal tract, or skin. This study provides more evidence that patients with EGPA may exhibit different features of disease based on their ANCA status.

SUN-375 REAL WORLD CLINICAL OUTCOMES IN ANCA ASSOCIATED VASCULITIS (AAV) – UNMET NEEDS IN ACHIEVING AND SUSTAINING REMISSION AND AVOIDING CUMULATIVE ORGAN DAMAGE

AAV is a severe systemic vasculitis and rapid induction of remission is desirable and then maintenance treatment required to avoid relapse. However, response to therapy can be variable and treatment-related infections and adverse events are common leading to high acute mortality and long term organ damage. This retrospective study examined AAV remission and maintenance and AEs in incident and maintenance AAV patients managed in routine clinical practice Two retrospective real world studies were performed. Firstly, 929 incident AAV patients (54% of patients had granulomatosis with polyangiitis, remainder microscopic polyangiitis) from 4 EU countries (399 physicians) were diagnosed between 2014-17 and data collected at baseline, 1, 3, 6 and 12 months following start of induction therapy was reviewed. Secondly 1478 AAV patients (49% of patients had granulomatosis with polyangiitis) from 4 EU countries (493 physicians) who completed induction therapy for organ/life threatening AAV and initiated maintenance therapy between 2014-16 were studied. Data were collected at the time maintenance was determined to begin by the physician and then at 6, 12, 18 and 36 months Incident patients had mean age of 56.82 yrs (SD 14.2) with 53.7% male. Birmingham vasculitis activity score (BVAS) was used in only 12%. Induction therapy varied with 59% receiving CYC, 24% RTX and 3% a combination, whilst 83% received GCs. As BVAS was not used routinely, clinical response was assessed as full (no AAV activity and GC taper on track), partial (reduction in AAV activity), or no response (no improvement in AAV). Table 1 shows that response rate varied, most patients required longer term GCs and adverse therapy impact was common. Full response at 1 month was associated with good 12-month outcomes (81% full response) whereas partial response at 1 month (56%) was less favourable (58% full response at 12 months). 78% of patients did not relapse over 12 months, 17% had 1 relapse, 3% had 2 relapses, and 2% had more than 2 relapses. Maintenance patients had mean age 54 years with 56% male. 49% were studied from incident and 51% from time of a relapse. Physicians defined maintenance start with mean of 5.7 months from induction start on basis of fixed time point 40%, start of new drug for maintenance 26%, reaching full remission 26% and no specific criteria 8%. Treatment at start of maintenance as GCs 69%, Azathioprine 34%, Rituximab 21%, Mycophenolate 17%, Cyclophosphamide 16% and methotrexate 8%. After 36 months (Table 2) of maintenance, 81% were alive and in remission but 10% had major relapse requiring re-induction therapy and left follow up, 4% died (in 2/3 of cases at time of relapse). AEs and infection were common and many patients required long term GCs Response to remission induction therapy in AAV patients is still variable with many patients not achieving a full response over the first 12 months of therapy. Infections and treatment-related adverse events are common, especially in the first 3 months. Maintenance period is variably defined but typically around 6 months when many patients are still not fully in remission and the majority are still receiving GCs. Relapse, major and minor, are still common and patients are still faced with treatment AE burden. Unmet need in AAV remains high and there is a need for targeted therapy to improve clinical outcomes.

Severe strongyloidiasis and systemic vasculitis: comorbidity, association or both? Case-based review

A possible association between strongyloidiasis and systemic vasculitis is rarely reported in the literature. We report the case of a patient with severe strongyloidiasis and an angiographic finding consistent with polyarteritis nodosa. Diagnosis of strongyloidiasis was made by finding of larvae and adult parasites in samples of the upper gastrointestinal tract mucosa and stool. The patient was treated with albendazole, ivermectin and corticosteroid withdrawal. This therapy led to the resolution of symptoms, with repeated stool samples negative for S. stercoralis. However, the clinical course was complicated with pulmonary tuberculosis. Despite tuberculostatic therapy and supportive measures, a lethal outcome occurred. The report is followed by a focused review of the available literature on the association of strongyloidiasis and systemic vasculitis.

PSY4 - RESPONSE TO INDUCTION THERAPY, TREATMENT ADVERSE EVENTS AND HEALTHCARE RESOURCE USE IN INCIDENT ANCA-ASSOCIATED VASCULITIS (AAV) PATIENTS

AAV is a severe systemic vasculitis and rapid induction of remission is desirable. Therapy is a combination of high dose glucocorticoids (GC) with rituximab or cyclophosphamide. Treatment-related adverse events (AE) are common and contribute to long term organ damage and acute morbidity. This study examined clinical outcomes, AEs and resource use in incident AAV patients in routine clinical practice. 929 incident EU AAV patients were diagnosed between 2014-17. Data at baseline, 1, 3, 6 and 12 months following commencement of induction therapy were reviewed. Mean age was 56.82 years with 53.7% male. Comorbidities were common (hypertension 45%, diabetes 18%, COPD/asthma 15%, coronary arterial disease 10%, arthritis 9%, osteoporosis 7% and cardiac failure 6%) with only 32% having none. Resource use was significant with 68.9% of patients hospitalized (mean length of stay 17 days including 3 ICU days). Clinical response was assessed as full (no vasculitis activity and GC taper on track), partial (reduction in vasculitis activity), or no response (no improvement in vasculitis). Full response was variable (18%, 43%, 61%, and 59% at 1, 3, 6, and 12 months, respectively) and AEs common (corresponding patients experiencing at least one AE 45%, 42%, 35% and 30%). Many patients experienced at least one infection (27%, 28%, 23% and 20%); some required hospitalization due to vasculitis or infection (11.3% median 9.1 days, 9.8% 7.6, 8.3% 7.4, 5.7% 10.3). 16% commenced dialysis in month 1, 3% at 3 months, 1% at 6 and 12 months Remission induction therapy has a variable response rate. Infections and AEs are common, especially in the first 3 months and there is significant healthcare resource use. New targeted therapy options are needed to improve response rates in AAV and reduce burden of therapy.

Raynaud’s Phenomenon of the Tongue

An elderly patient with multiple autoimmune conditions was found to have intermittent unilateral and bilateral tongue cyanosis consistent with Raynaud’s phenomenon in the setting of severe systemic vasculitis. Her presentation, laboratory findings, and clinical course are reviewed. A 71-year-old woman with a history of multiple autoimmune conditions including Sjogren’s syndrome, mixed connective tissue disease, and autoimmune hepatitis was admitted to hospital for complications during treatment for marginal cell lymphoma. She …

Diagnosis and treatment of cutaneous leukocytoclastic vasculitis

We review the terminology used in the context of cutaneous vasculitis with discussion of cutaneous leukocytoclastic vasculitis, its clinical approach, differential diagnosis and treatment algorithm. Cutaneous vasculitis encompasses a wide spectrum of conditions of very different severity and urgency, from limited skin disease to severe systemic life-threatening vasculitis. This review will empower the reader with tools for rapid evaluation of patients suspected of cutaneous vasculitis such as a correct interpretation of skin biopsies, an effective high yield laboratory and imaging testing approach, a simple practical evaluation procedure to ensure that severe organ involvement by a systemic vasculitis is not missed, as well as an easy diagnostic algorithm for identification of the cause of vasculitis.

Kawasaki Disease Complicated With Reversible Splenial Lesion and Acute Myocarditis

Kawasaki disease, a systemic vasculitis of unknown etiology, develops frequently in infants and demonstrates a variety of clinical symptoms during the disease course. The most important complication, coronary artery lesions, is found in 15-25% of untreated patients. Meanwhile, acute myocarditis, another complication that can occur during the acute phase of severe systemic vasculitis, has been found in more than 50% of affected individuals when asymptomatic cases are included. However, cases that require treatment are rare as reported by Yoshikawa et al. (Circ J 70:202-205, 2006). As for neural complications, aseptic meningitis is well known, but it is extremely rare for these patients to develop encephalitis or encephalopathy as reported by Imai et al. (Jpn Soc Emerg Pediatr 8:50-55, 2009). Recently reported magnetic resonance images (MRIs) have shown reversible lesions in the median splenium of patients complicated with encephalitis or encephalopathy. Reversible lesions have also been observed after the administration of an antiepileptic agent, drastic weight loss, and development of metabolic abnormalities as reported by Massimo et al. (Neuroradiology 49:541-544, 2007) and Tada et al. (Neurology 63:1854-1858, 2004). Aggressive therapy for such lesions is not considered necessary because most disappear without neurologic aftereffects. However, the clinical significance and pathogenesis of the condition remain largely unknown. We present the first known report of a Kawasaki disease case complicated with acute myocarditis and mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). These findings may be valuable for the diagnosis and treatment of affected patients.

ICU by Default: Consequences and Solutions to Deferred Palliative Care

1.Describe barriers to palliative care consults in the acute-care setting through case report.2.Discuss effective solutions to upstream palliative care in advanced disease. I. Background. Intensive care unit (ICU) episodes for patients with advanced or terminal disease can occur if goals of care are not clearly defined or discussions of goals deferred entirely. These patients may experience symptom distress and ICU transfers that are not beneficial or consistent with their preferences. The competing priorities of comfort and cure and the misunderstanding about the benefits of palliative care are associated with inadequate use of palliative care. A solution to deferred palliative care and non-goal oriented ICU trials is a new model of integration used at our center, palliative care–acute care, or PAC rounds. The purpose of these weekly rounds is to identify patients with palliative care needs earlier in the hospital stay. Patients who meet specific screening criteria are presented to a multidisciplinary team. This case report affords an excellent opportunity to examine the consequences of deferral of palliative care and the impact of PAC rounds. II. Case Description. A 65-year-old female with severe systemic vasculitis underwent an extensive diagnostic evaluation. During this time, she had significant symptom burden including severe pain, delirium, dyspnea, constipation, and had extensive cutaneous involvement in both lower extremities. Renal, cardiology, and dermatology were consulted. The patient was transferred to the ICU after an episode of desaturation thought to be due to opioids. The patient remained a full code until the palliative care consult service coordinated a multidisciplinary meeting with the patient's spouse to clarify goals of care. The patient died within 2 days of ICU transfer on day 10 of hospitalization. III. Conclusion. Often the goal in acute illness is aggressive treatment or extensive diagnostic evaluation and palliative care goals may be perceived as conflicting. This case report illustrates the poor outcomes that can result if goals of care are not clearly elucidated but also an opportunity to upstream palliative care. Structure and Processes of Care

Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group

BACKGROUND: Henoch-Schonlein purpura (HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It has been extensively studied in children, but in adults, its natural history is much less known. OBJECTIVES: to report a series of patients with HSP presenting in their adulthood. PATIENTS AND METHODS: the Minas Gerais Vasculitis Study Group´s Members (MGVSG) were invited to report patients with HSP who appeared in their adulthood. A standardized retrospective chart review was done. RESULTS: eleven patients, two male and nine female, age 39.4 +/- 20.1 yearsold were studied. Nine patients presented purpura, seven presented arthritis, four patients had gastrointestinal involvement and ten patients (91%) presented glomerulonephritis (GN). Eight patients were subjected to renal biopsies. Six of them presented endocapillary proliferative GN and only two of them had minimal mesangial proliferation. In the other three patients, HSP was confirmed by skin biopsies. All patients received steroids, in five of them steroids were combined with other immunosuppressive agents. After a follow-up of 39.0 +/- 64.6 months, four patients (36%) presented impairment of renal function, but only one (9%) developed end stage renal disease and was successfully appeared to renal transplantation. At the end of follow-up, seven patients (64%) are in complete remission and four in partial remission. CONCLUSION: in adulthood, HSP represents a distinct clinical syndrome with a higher frequency of renal involvement and more severe systemic vasculitis. Nevertheless, the final outcome in this series was as good as reported in children, maybe due to aggressive immunosuppressive therapy.

Severe systemic vasculitis in a neonate

A full-term male newborn, the child of a healthy Swiss woman with normal urinalysis and negative tests for hepatitis B, hepatitis C and human immunodeficiency virus, was referred for surgical repair of esophageal atresia and distal tracheoesophageal fistula at the age of 2 days. Three days later, the diagnosis of anastomotic leak and infected right-sided pneumothorax was made, which required parenteral antimicrobial treatment with clindamycin and amoxicillin based on positive cultures for both viridans streptococci and coagulase-negative staphylococci. In spite of that the child developed a severe pleural empyema, which was treated with thoracotomy and decortication. The antimicrobial medication was continued up to the age of 21 days. Two days later, the condition of the child deteriorated, as indicated by the appearance of tachypnea, progressive cyanosis, and reduced diuresis. Chest x-ray findings disclosed intraalveolar fluid accumulation. Laboratory values were as follows: hemoglobin, 91 g/l (reference range, 100–150 g/l); white blood cell count, 19.8×10/l (reference range, 5.0–12.5×10/l); platelets, 39×10/l (reference range, 150–300×10/l); C-reactive protein, 118 mg/l (reference range, ≤5 mg/l); creatinine, 92 μmol/l (reference range, ≤47 μmol/l). A blood film disclosed a moderate eosinophilia of 3.9×10/l (reference range: ≤0.8×10/l). Urinalysis disclosed severe proteinuria, hematuria and red blood cell casts. Two days later, a generalized maculopapular rash was noted. Antineutrophil cytoplasmic and antinuclear autoantibodies, and antibodies against the Ro and La particle were not present in either the patient or his mother. The tentative diagnosis of systemic vasculitis was confirmed by a skin biopsy demonstrating a characteristic leucocytoclastic vasculitis with marked eosinophilia (Fig. 1). Immunofluorescence studies with antisera against immunoglobulins were not performed. Medical treatment with methylprednisolone 3 mg/kg body weight daily was followed within 24 hour by polyuria Eur J Pediatr (2007) 166:381–382 DOI 10.1007/s00431-006-0242-9