ANCA-Associated Vasculitis Following Johnson and Johnson COVID-19 Vaccine

Antineutrophil cytoplasmic autoantibodies associated vasculitis(AAV) is characterized by antibodies against antigens in cytoplasmic granules of neutrophils and predominantly affects small vessels. AAV after COVID-19 mRNA vaccination has been reported. We report rare case of AAV in a patient who presented with rapidly progressive glomerulonephritis(RPGN) after Johnson & Johnson vaccine administration.

The misleading laboratory results: Renal impairment associated with high anti myeloperoxidase titer (anti-MPO) does not always mean ANCA vasculitis

Introduction: Antineutrophil cytoplasmic autoantibodies (ANCAs) are crucial in the pathogenesis of ANCA-associated glomerulonephritis. Although highly specific for this disease, these antibodies can appear in healthy individuals or occur in other settings such as malignance, other inflammatory disease, or drugs. Renal biopsy remains essential for diagnosis and as a prognostic tool. Failing to perform a biopsy risks an incomplete evaluation or misdiagnosis. Clinical case: A 60-year-old male known to have had previous normal kidney function was admitted with generalized pitting edema, nonpruritic erythematous rash, and decreased urine output. He had been recently started on clozapine for depression. Laboratory tests revealed nephrotic syndrome with massive proteinuria (11g/day), hematuria and elevated serum creatinine that reached a maximum of 4.9 mg/dl. The only positive finding was a high titer of myeloperoxidase (MPO) ANCA antibody (111 UI/ml). Clozapine was discontinued, and he was started on conservative management of the nephrotic syndrome. The rash disappeared within one week. Kidney biopsy showed cellular variant focal segmental glomerulosclerosis (FSGS) with mild acute tubular injury. The patient was treated with oral prednisolone with complete remission. Repeat MPO-ANCA was negative after one month. Discussion: We present a challenging case that highlights the importance of kidney biopsy for accurate diagnosis with major impact on treatment and prognosis. This case also underlines the presentation and evolution of FSGS cellular variant, a rare and poorly understood disease. The relationship between skin rash with high MPO titer and the administration of clozapine with clinical remission after its discontinuation favors the hypothesis of drug associated ANCA-vasculitis.

Treatment updates in Antineutrophil Cytoplasmic Autoantibodies (ANCA) vasculitis

Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis is a small vessel vasculitis (SVV) resulting in inflammation of small- and medium-sized blood vessels. Since the initial description of SVV, there have been tremendous advances in our understanding of the pathogenesis. Over the last decade, we have made significant progress in understanding the pathogenesis and improving the treatment and prognosis of patients with ANCA vasculitis. Patient and renal survival has improved, and treatment is moving towards individualizing care, minimizing severe adverse events, and preventing relapse. This review focuses on treatment updates in ANCA vasculitis, duration of therapy, and management of relapses. We also describe the existing treatment protocols utilized at our institution.

P0335PULMONARY HEMORRAHGE IN IG A NEPHROPATHY: A RARE MANIFESTATION

Background and Aims Ig A nephropathy is the most frequent cause of glomerulonephritis worldwide. Renal-limited disease is the habitual clinical form of the disease. Pulmonary involvement may also be seen in rare cases. Capillaritis with pulmonary hemorrhage is the most frequent pulmonary involvement and is lifethreatening. We reported the case of patient presenting a rapidly progressive glomerulonephritis with pulmonary hemorrhage revealing Ig A nephropathy. Method A case report Results A 23 year-old man admitted for a rapidly progressesive glomerulonephritis with sever hypertension. He presented a respiratory distress with hemoptysis. At biology serum creatinine was at 2200µmol/l (eGFR = 2.4 ml/min) and Hemoglobin was at 5g/dl. Immunoassays of anti-glomerular basement antibodies and antineutrophil cytoplasmic autoantibodies were negatives. Computed tomography scan showed diffuse intra alveolar hemorrhage associated with pleural effusion. Bronchoalveolar lavage confirms this diagnosis. Renal histology showed Ig A nephropathy. Treatment consisted of daily depleted haemodialysis with blood transfusion and corticosteroids associated with monthly pulses of cyclophosphamide. His hemoptysis gradually resolved with a stable hemoglobin at 11 g/dL. However, renal failure persisted requiring maintenance hemodialysis regularly. Conclusion Pulmonary hemorrhage is a rare but serious complication of Ig A Nephropathy which is associated with significant morbidity and mortality. The exact cause for this “extra renal bleed” in Ig A Nephropathy is not known but it possibly represents IgA capillaritis. Though no evidence-based treatment protocol exists, therapeutic options includes steroids, plasmapheresis, cyclophosphamide and rituximab.

MIKROSKOPINIS POLIANGITAS. KLINIKINIS ATVEJIS

Reikšminiai žodžiai: mikroskopinis poliangitas, ANCA, aortos aneurizma, širdies tamponada. Pristatome klinikinį atvejį 68 m. moters, kuri pirmą kartą kreipėsi į gydytojus 2003 m. dėl nepastovių, migruojančių sąnarių skausmų. 2008 m. dėl inkstų funkcijos sutrikimo atlikta biopsija, nustatytas židininis pusmėnulinis sklerozuojantis glomerulonefritas, asocijuotas su antineutrofilinių citoplazminių antikūnų (angl. antineutrophil cytoplasmic autoantibodies) buvimu. Diagnozuotas mikroskopinis poliangitas, paskirta prednizolono ir ciklofosfamido pulsterapija. Praėjus keturiems mėnesiams po prednizolono nutraukimo, atsirado plaštakų ir kelių sąnarių skausmai. Skirtas gydymas prednizolonu, azatioprinu, fosinopriliu (hipertenzijos korekcijai), vaistų dozės buvo koreguojamos atsižvelgiant į ligos eigą ir inkstų funkcijos rodiklius. 2014 m. diagnozuota antrinė sensomotorinė distalinė galūnių polineuropatija. 2015-05-10 pacientė kreipėsi dėl stiprių nugaros skausmų, kurie prasidėjo prieš 3 mėnesius. Įtarus aortos aneurizmą, atlikta krūtinės ląstos kompiuterinės tomografijos angiografija, nustatyta aortos kylančiosios dalies aneurizma be disekacijos požymių ir daugybiniai kompresiniai slankstelių lūžiai (krūtininiai 6–10, 12). Dėl sunkios būklės kardiochirurginė intervencija kontraindikuotina. 2015-05-11 diagnozuotas galvos smegenų infarktas, somnolencija, lengva tetraparezė. 2015-06-04 nustatyta kairės kojos ūminė giliųjų venų trombozė. 2015- 06-23 plyšo kylančiosios aortos aneurizma, susidarė hemoperikardas ir išsivystė širdies tamponada, gaivinimo priemonės neefektyvios – konstatuota pacientės mirtis. Šiame straipsnyje pateikiame klinikinio atvejo analizę – pacientės, sergančios mikroskopiniu poliangitu, kuris pasireiškė sunkiomis ir retomis komplikacijomis.