Reactivation of IgA vasculitis following COVID-19 vaccination

A 23-year-old man presented to the acute assessment unit with acute-onset haematuria within 24 hours of receiving his second dose of the Pfizer-BioNTech COVID-19 vaccine. He had been diagnosed with IgA vasculitis 8 months previously. IgA vasculitis is an autoimmune condition characterised by palpable purpura affecting the lower limbs, abdominal pain, arthralgia and renal disease. He was diagnosed with an acute exacerbation of IgA vasculitis and was discharged with oral prednisolone. Reactivation or first presentation of IgA vasculitis is a rare but increasingly recognised complication of COVID-19 vaccination. This is an important new differential in the assessment of patients with haematuria following COVID-19 vaccination.

POS0290 PREDICTING RISK FACTORS OF MALT LYMPHOMA IN SJÖGREN’S SYNDROME

Background:Primary Sjögren Syndrome (SS) is a slowly progressive systemic autoimmune disease complicated by lymphoma, with mucosa associated lymphoid tissue (MALT) type being the most common lymphoma form. Several predictors related to pSS associated lymphomas have been described, but there are no studies focusing on specific risk factors for the MALT histologic subtype.Objectives:To identify predictors at SS diagnosis for MALT lymphoma development in pSS patients using simple clinical features.Methods:From 815 SS patients of a single center fulfilling the 2016 ACR/EULAR criteria, those with subsequent development of MALT lymphoma according to the 2016 WHO classification were identified and matched in 1:2 ratio, with non-lymphoma SS control patients according to age, disease duration from SS diagnosis and gender. Lymphoma patients diagnosed within a year from SS diagnosis were excluded from the current study. Clinical, laboratory, histologic data as well as the ESSDAI scores at the time of SS diagnosis were recorded and compared between lymphoma and non-lymphoma patients. Independent lymphoma predictors were identified by a data driven Fast Correlation Based Feature selection (FCBF)/Logistic Regression (LR) algorithm.Results:A unified dataset of 57 MALT lymphoma patients and 114 non lymphoma controls along with 39 features/variables was generated. The median age of SS diagnosis and the disease duration from SS diagnosis to lymphoma diagnosis (lymphoma group) or last follow up (control group) was 50,5 years old (range 25-77) and 7 years (range 0- 30) for the control group and 50 years old (range 24-70) and 8 years (range 1 -30) for the lymphoma group, respectively. MALT lymphoma patients presented more frequently with palpable purpura (23,2% vs 5,3% p=0,001), cryoglobulinemia (30,2% vs 1,6% p<0,0001), low C4 serum levels (62,9% vs 32,1% p=0,0003), rheumatoid factor (76,9% vs 56,1% p=0,01), anti La/SSB antibodies (33,9% vs 50,8% p=0,049) and higher median ESSDAI score (5 vs 2, p<0,0001). In contrast, autoimmune thyroiditis was more prevalent in controls (48,2% vs 18,6%, p=0,004). The FCBF/LR model revealed cryoglobulinemia (p=0,03) and ESSDAI at SS Diagnosis (p<0,001) as the only independent lymphoma predictors.Conclusion:MALT is the predominant pSS related lymphoproliferative histologic type, associated with systemic disease activity and vasculitic manifestations at SS diagnosis. Cryoglobulinemia and ESSDAI score were proven independent risk factors for MALT lymphoma development.Table 1.An FCBF-based multivariable logistic regression analysis results for investigating risk factors for MALT lymphoma developmentProminent featureRegression coefficientOdds ratiop-valueCI lowCI upperCryoglobulinemia1.675.3420.033*1.1824.327Total ESSDAI at diagnosis0.281.318<0.001*1.2081.439Kidney involvement0.071.0690.50.1010.351•< 0.05 (95% confidence interval). The rest of the features that participated in the analysis include the following: Palpable purpura, Low C4, Salivary gland enlargement, Lacrimal gland enlargement, ANA Titers, RF, Focus score at Sjögren diagnosis, PNS involvement, Anti-La, Disease duration from SS onset to SS diagnosis, Neutrophils<1500, Autoimmune thyroiditis, Lung involvement – interstitial disease Type, Lymphocytes<1000, Lymphadenopathy fixed, Arthralgias, Dry eyes, Raynaud, Gender, Age at Sjögren diagnosis, Dry mouth, Aca, Hb<12.5 g/dL, ANA, Disease duration from SS Diagnosis to Lymphoma diagnosis or last follow up, Anti-Ro, Arthritis, WBC<4000/μL, Lung involvement bronchocentric disease, Interstitial renal disease, PLTs<100.000/μL, Liver involvement-PBC, Liver involvement–autoimmune hepatitis, CNS involvement.•AUC=0.78Disclosure of Interests:None declared

Stereotactic Radiotherapy in the Treatment of Paraneoplastic Vasculitis in Oligometastatic Renal Cell Carcinoma

Approximately 20% of renal cell carcinoma (RCC) is diagnosed because of paraneoplastic manifestations. RCC has been associated with a large variety of paraneoplastic syndromes (PNS), but it is rarely associated with PNS vasculitis. We present a case of a previously healthy male who presented with systemic vasculitis; bitemporal headaches, diplopia, polyarthritis, palpable purpura, tongue lesion, peri-orbital edema, scleritis, chondritis and constitutional symptoms. He was subsequently found to have oligometastatic RCC. Both his primary lesion and site of oligometastasis were treated with stereotactic radiotherapy (SBRT) and resulted in the resolution of his vasculitis, as well as sustained oncologic response. This is the first case to demonstrate that effective sustained treatment for PNS vasculitis due to oligometastatic RCC is possible with SBRT.

Mediterranean spotted fever associated with leucocytoclastic vasculitis and acute pancraeatitis

A young healthy gardener became febrile with abdominal pain, nausea, vomiting and diarrhoea followed by palpable purpura, mostly on the legs and buttocks with associated arthralgia. Dehydration, azotemia and hyponatraemia resolved with fluid replacement. Tests demonstrated acute pancreatitis, hepatitis, thrombocytopenia, microscopic haematuria and proteinuria. He improved with doxycycline, but bipedal pitting oedema and punctate rash involving the soles/hands appeared. Microbiological tests revealed positive IgM and IgG serology for rickettsiae spotted fever. Skin biopsy of the purpura confirmed leucocytoclastic vasculitis, positive for Rickettsiae conorii by PCR amplification. Palpable purpura is a rare important manifestation of Mediterranean spotted fever (MSF), due to either secondary leucocytoclastic vasculitis or associated Henoch-Schonlein purpura (HSP), which best explains the distribution of the rash, arthralgia, gastrointestinal symptoms, and microhaematuria not usually seen in R. conorii infections. Likewise, the patient’s acute pancreatitis may be interpreted as a rare presentation of HSP or a seldom-encountered feature of MSF.

Autoimmune syndrome induced by cosmetic injection of hyaluronic acid

Autoimmune/inflammatory syndrome induced by adjuvant (ASIA) is a condition in which exposure to an adjuvant leads to an aberrant immune response resulting in signs and symptoms of autoimmune diseases. We present the case of a patient with arthritis and palpable purpura after hyaluronic acid lip injection.

Vasculitis and Vasculopathies

Our organism, as complex as it is, needs a giant vascular network to deliver nutrients to all cells, so vasculopathies and vasculitis are diseases present in all medical specialties. The skin and subcutaneous cellular tissue are irrigated by a vast vascular network, with cutaneous involvement related to these frequent pathologies. These can be restricted to the integumentary system or be part of systemic diseases with cutaneous manifestations, which make them of great interest to dermatologists. They can affect any caliber of vessels and present with several dermatological manifestations such as erythema, livedo reticularis, palpable purpura, nodules, ulcers, urticaria, hemorrhagic blisters, gangrene and other manifestations that can be isolated or associated with systemic signs and symptoms. However, there is no worldwide consensus regarding the classification of vasculitis, and the classification proposed in this chapter is based on the International Chapel Hill Conference Nomenclature of Vasculitides 2012, which is based on the size of the vessels. The purpose of this chapter is to compile a review of the most current treatments for these conditions.

Acute appendicitis as the first manifestation of HenochSchönlein purpura

Henoch-Schönlein purpura (HSP) is a small vessel systemic vasculitis. Typical symptoms include palpable purpura, joint pain, and abdominal pain. Most cases improve after a few weeks, not requiring any treatments other than symptom control. Acute abdomen resulting from vasculitis is very rare and should be treated as a surgical emergency. We report the case of a 9-year-old boy with acute gangrenous appendicitis as the first manifestation of HSP.

Henoch-Schonlein Purpura with hematochezia in an adult patient

Henoch-Schönlein purpura (HSP) is a self-limiting vasculitic disease, commonly seen in children. It is characterized by palpable purpura, arthritis, hematuria and abdominal pain. Hematochezia is an uncommon symptom. The patient being reported is an adult male who presented with vomiting, abdominal pain and hematochezia, followed by appearance of palpable purpura and arthritis. He was diagnosed to have HSP. Hematochezia as one of the initial presentations of HSP in adults is an uncommon scenario.

AB0463 ETIOLOGY OF PALPABLE PURPURA; A SINGLE CENTER EXPERIENCE

Background:Purpura are lesions that occur after bleeding on the skin, mucous or serosal surfaces. Purpura can be classified into 2 subgroups; which are linked to thrombocytopenia and non-trombocytopenic purpura. While thrombocytopenic purpura often occurs due to a hematological disease; in non-trombocytopenic purpura etiological causes are very variable, and systemic vasculitis has an important place among them.Objectives:The demographic features of the patients applying with purpura and the underlying causes and diseases were aimed to be revealed.Methods:44 consecutive patients (22 women, 22 men) who were admitted to the hospital in the last 6 months, due to purpura were evaluated. Average age of patients was 49.6 ± 19.6 years. Patients were questioned about a recent infection, drug use, concomitant or underlying diseases. The serological tests and other laboratory tests for etiology were performed and biopsy was taken from the skin lesions which are appropriate.Results:While 4 (9%) patients had widespread purpura on the trunk-upper-lower extremities, in 22 (50%) patients purpuric lesions were limited only to the lower limb. The period between the onset of the first symptom and the admission to the hospital was longer than 4 weeks in 35 (79.5%) patients, and shorter than 1 week in 9 (20.5%) patients. 24 (54%) patients had an anamnesis of infection 2-3 weeks before purpura, and 20 (45%) patients had an anamnesis of drug use. The most common accompanying symptom was abdominal pain and was present in 15 (34%) patients. Biopsy was performed from the skin lesion in 37 patients. Histopathological examination of all was compatible with leukocytoclastic vasculitis. In indirect immunofluorescence staining, 17 were found to be IgA positive. 2 (4.5%) patients were diagnosed PR3-ANCA positive granulomatosis with polyangiitis. 1 patient had Hepatitis B virus infection was detected in 1 patient (2.2%), HIV infection was detected in 1 patient (2.2%) and malignancy was detected in 1 patient (2.2%).Conclusion:In our study, the most common reason was found as IgA vasculitis in patients presenting with palpable purpura. Although vasculitic involvement was limited to the skin in most patients, organ-threatening systemic vasculitis was detected in a few patients. Patients applying with Purpura should be questioned for infection and drug use, should be examined for underlying diseases including systemic vasculitis, and closely monitored for organ involvement.Disclosure of Interests:None declared