278 Cardiac involvement in a 23 years old patient with granulomatosis with polyangiitis (GPA)

Aims Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis, which could potentially affect any organ system. However, there have only been a few reports on cardiac involvement. In fact, it most commonly involves the sinuses, lungs, and kidneys with necrotizing granulomatous vasculitis. In 12% of a large series of patients with GPA there was cardiac involvement, largely manifested by pericarditis and coronary arteritis. Methods and results We describe a rare case of a 23-year-old girl, with no pathological history, at exception of a recent flu-like syndrome for which she carried out the search for SARS-CoV-2 RNA through nasopharyngeal swab, results negative. After a month, she went to the emergency department for a syncopal episode and subsequent head trauma. On this occasion, echocardiogram performed showed the presence of left ventricular systolic dysfunction due to hypokinesia of the middle distal segments; CT angiography of the chest revealed the presence of pulmonary embolism. For this reason, the patient was admitted to the cardiac intensive care unit, where EKG shown anterolateral myocardial infarction with ST elevation and immediately was performed coronary angiography, that evidenced two-vessel disease, with subsequent ineffective attempt to angioplasty. Due to the intercurrent appearance of hyposthenia and paraesthesia in the left upper limb, CT angiography of the brain was performed with detection of lower right pre central frontal hypodensity, suspected for recent ischaemic lesion and hypodensity of the right carotid artery as recent thrombosis. In light of the multi-organ involvement of ischaemic nature and the young age of the patient, rheumatological evaluation was carried out, with execution of a laboratory tests that showed the presence of positivity for ANCA anti-PR3 antibodies, on the basis of which was diagnosed GPA, and rituximab therapy was immediately initiated, with clinical benefit. Conclusions Cardiac involvement of GPA was first reported by Wegener in 1936. Classical or generalized GPA is characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis. Widespread disseminated vasculitis involving both small arteries and veins occurs to a greater or lesser degree as the disease progresses. A localized form of GPA limited primarily to the upper and lower respiratory tracts has been described. Despite histopathological diagnosis of GPA, with autoantibodies against to circulatory neutrophilic cytoplasmic antigens, we can diagnose GPA easily and early. GPA must be kept in mind as the differential diagnosis of new onset cardiomyopathy, especially in the existence of pulmonary and renal pathologies. The clinical presentation of GPA can be so diverse that the list of differential diagnoses is vast, ranging from infections (fungal, bacterial, and mycobacterial) to other vasculitides, including Henoch–Schönlein purpura, sarcoidosis, Behcet syndrome, and malignancies. Despite that involving the heart is well described, significant cardiac complications occurring during the course of the disease are rare.

Gastrointestinal Hemorrhage in Patient with Granulomatosis with Polyangitis

Granulomatosis with polyangitis (GPA) is characterized by a necrotizing granulomatous vasculitis of small arteries and veins. It most commonly affects the upper and lower respiratory tract and kidneys. However, other organs including the gastrointestinal tract can be affected. Gastrointestinal manifestations of GPA are rare and can include ischemia, bowel infarction, and perforation. Hemorrhage is an extremely rare presentation of GPA. We present a case of a woman with GPA and pulmonary renal syndrome on treatment who presents with severe gastrointestinal hemorrhage.

Glucocorticoid Effects on Tissue Residing Immune Cells in Giant Cell Arteritis: Importance of GM-CSF

Giant cell arteritis (GCA) is a systemic granulomatous vasculitis clinically characterized by a prompt response to glucocorticoid therapy. Dendritic cells (DCs) play a central role in the pathogenesis of the disease and are increased in temporal arteries from GCA patients. The aim of this study was to determine the effects of glucocorticoid therapy on granulomatous infiltrates and on peripheral DCs of GCA patients. Immunohistochemical staining of temporal artery specimens from 41 GCA patients revealed a rapid reduction of the number of DCs after initiation of glucocorticoid treatment. TUNEL staining was performed to quantify apoptotic S100+ DC, CD3+ T cells, and CD68+ macrophages in the granulomatous infiltrates. An increase of apoptotic cells up to 9 ± 2% after 4–5 days of glucocorticoid therapy and up to 27 ± 5% (p < 0.001, compared to earlier timepoints) after 6–10 days was detected. A decrease of CCL19 and CCL21 expression was observed after starting glucocorticoid therapy. Granulocyte-macrophage colony-stimulating factor (GM-CSF) expression also significantly decreased under glucocorticoid therapy. No GM-CSF expression was detected in the control specimens. Glucocorticoid therapy leads to a rapid, time-dependent reduction of DCs in temporal arteries from GCA patients and reduction of mediators for cell migration. Our data suggest GM-CSF as a novel therapeutic target of GCA.

Kidney complications following COVID-19 vaccination; a review of the literature

Objective: To review the reported cases of kidney injury following vaccination for coronavirus disease 2019 (COVID-19) with a focus on renal pathology. Methods: We searched for case reports of kidney complications after COVID-19 vaccine in PubMed. Results: A total of 36 articles including 49 case reports were reported. These included minimal change disease (n=17), IgA nephropathy (IgAN) (n=15), IgA nephritis/vasculitis (n=5), ANCA glomerulonephritis/vasculitis (n=5), anti-glomerular basement membrane (GBM) nephritis (n=2), and 1 case of each granulomatous vasculitis, acute tubulointerstitial nephritis, scleroderma renal crisis, IgG4-related disease nephritis, and primary membranous nephropathy (MN). Conclusion: We give an overview of the reported cases of post-COVID-19 renal complications. Further investigations of the underlying pathogenesis of post-COVID-19 vaccination renal adverse events are required, as prompt workup, diagnosis, and treatment of patients with renal complications may lead to complete remission, prevent kidney failure, and long-term complications such as end-stage renal disease (ESRD). However, these complications are overall extremely rare and the benefit of vaccination outweighs the potential risks.

A Case of Granulomatosis with Polyangiitis with Various Breast Lesions as the Initial Symptoms: A Case-Based Review

A 44-year-old woman presenting with pus-like discharge from the nipples visited our hospital for scleritis. Subcutaneous induration and ulceration were found on her breast. She was diagnosed with granulomatosis with polyangiitis (GPA) considering scleritis, sinusitis, cutaneous granuloma formation, and antiproteinase 3-antineutrophil cytoplasmic antibodies and was successfully treated with glucocorticoids. Fifteen months later, she developed pulmonary consolidation and a right breast nodule. Biopsies of the breast nodule showed granulomatous vasculitis, and she was treated with rituximab. While breast involvement in GPA is rare, unilateral breast mass is a typical clinical feature; thus, GPA should be considered in such cases.

Challenges in the Diagnosis of a Case of Granulomatosis with Polyangeitis

Granulomatosis with polyangiitis is a multisystem disease characterized by a necrotizing granulomatous vasculitis, typically associated with the presence of anti-neutrophil cytoplasmic antibodies. The lung is the most common organ involved in comparison to other vasculitis, but in the case presented by us the onset is otorhinolaryngology field, with a multitude of symptoms and immunological tests are initially negative. Over time, immunological tests become positive, but all this leading to a delay in diagnosis by 4 years and of course to a delay in treatment. In this paper we emphasize the usefulness of biopsy for diagnosis and use of anti-neutrophil cytoplasmic antibodies to classify the disease.

Posterior reversible encephalopathy and status epilepticus in Takayasu arteritis

Takayasu arteritis (TA) is a rare, systemic, chronic inflammatory condition causing granulomatous vasculitis of medium-sized and large arteries of unknown etiology. A possible relationship between Takayasu arteritis and tuberculosis has been suggested but not proven until now. Posterior Reversible Encephalopathy Syndrome (PRES) and seizure are rare complications. We report a case of Takayasu arteritis with lymph node tuberculosis presented with status epilepticus and Posterior reversible encephalopathy syndrome.

Cryopyrin-associated periodic syndrome: a treatable genetic inflammatory condition

A 20-year-old man presented with recurrent subdural haemorrhages on a background of progressive sensorineural hearing loss, juvenile idiopathic arthritis and intracranial hypertension of unknown cause. His mother had a similar previous history. They both had a persistently mildly elevated serum C reactive protein. Repeat lumbar punctures identified persistently elevated intracranial pressure and mild pleocytosis. A dural biopsy showed necrotising pachymeningitis with granulomatous vasculitis. The underlying cause in both patients was a cryopyrin-associated periodic syndrome. We discuss its varied phenotype and how clinicians need to be aware of this treatable genetic condition to facilitate early treatment and to prevent accumulation of disability.