Immune System Disorders

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  • Amal El-Ouakhoumi, Hajar Joulal, Jaouad Yousfi, Laila Benjilali, Mouna Zahlane, Lamiaa Essaadouni
    Views: 24 HTML: 0 PDF: 15

    Background: The psychiatric manifestations of Sjögren’s syndrome are often overlooked despite their prevalence. They can be revelatory of the disease and include anxiety, depression, dementia and, rarely, psychosis.
    Case description: We report a case of 18-year-old female in whom a major depressive syndrome revealed primary Sjögren’s disease, with a favourable outcome after treatment with rituximab.
    Conclusion: The diagnostic of Sjögren’s syndrome should be considered in patients who present with unexplained and refractory neuropsychiatric symptoms, even in the absence of sicca symptoms.

  • Josef Finsterer
    Views: 43 HTML: 7 PDF: 30
  • Fatih Kaya, Tarek Alsafdi, Manar Hussam Al-Suleh
    Views: 231 HTML: 54 PDF: 116

    The incidence of post-infectious autoimmune diseases has been on the rise following the COVID-19 pandemic. Recently, an autistic patient was admitted to the hospital presenting with a mild upper respiratory system COVID-19 infection. Months after recovery and polymerase chain reaction negativity, the patient developed HEp-2 cell positivity and presented with relapsing polychondritis (RP), a rare autoimmune disease. The mechanism of this autoimmune invasion is ultimately caused by activating a myriad of immune reactions. Lymphocytopenia almost always accompanies various clinical forms of COVID-19; however, it may drive the lymphocytopenia-induced proliferation of autoreactive T cells via the activation of interleukin-6 (IL-6). Moreover, high levels of neutrophils during infection promote autoimmune disease by releasing cytokine and chemokine cascades that accompany inflammation, and neutrophil extracellular traps regulating immune responses through cell–cell interactions. Furthermore, autism spectrum disorder patients display an altered immune system that includes an augmented inflammatory cytokine milieu leading to an increased pro-inflammatory Th1/Th2 ratio. In addition, the pathophysiology of RP is majorly associated with a cell-mediated immune reaction; thus, the predisposing exaggerated immune system of such patients must also be considered as a predisposing factor to the development of post-infectious autoimmune diseases.

  • Fatimaezzahra Bensalek, Hajar Joulal, Jaouad Yousfi, Mouna Zahlane, Leila Benjilali, Lamiaa Essaadouni
    Views: 193 HTML: 9 PDF: 92

    Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, characterised by multi-organ affections. Haematological involvement is a common manifestation of SLE, consisting of autoimmune peripheral cytopenia. Autoimmune myelofibrosis (AIMF) is a rare cause of cytopenia in SLE; it could precede or be concurrent with the diagnosis of SLE. There are few studies that describe this association.
    Case description: We report a case of AIMF revealing the diagnosis of SLE in a 34-year-old female, presented with episodes of gingival bleeding associated with peripheral inflammatory polyarthralgia, photosensitivity and deterioration of general condition. Clinical examination revealed a soft pitting oedema in the lower limbs. Laboratory investigations showed a pancytopenia, inflammatory biological syndrome, with positive 24-hour proteinuria and anti-native DNA antibodies. A bone marrow biopsy showed diffuse myelofibrosis associated with maturation disorders and no tumour infiltrate. Renal biopsy revealed proliferative glomerulonephritis class III with immune deposits.
    Conclusion: The association of AIMF with SLE has been rarely reported, and it could be another cause for cytopenia in SLE.

  • Benedetta Tomberli, Stefano Fumagalli, Tiziana Cristina Minopoli, Silvia Menale, Valentina Scheggi, Niccolò Marchionni
    Views: 119 PDF: 104 HTML: 10

    Intracoronary in-stent restenosis (ISR) is a phenomenon that generally occurs between 3 and 6 months after stent placement. With the introduction of drug-eluting stents (DES), the incidence of ISR has decreased but not disappeared. We report a case of reiterant in-stent restenosis of an 81-year-old female patient who underwent multiple percutaneous coronary intervention and two coronary artery bypass surgeries. ISR is possibly associated with extra-stent, stent-related and intra-stent factors. Here, we excluded the first two and focused on the intra-stent factors that seem more likely in our case. A challenging diagnostic workup led us to the hypothesis of a coronary vasculitis potentially triggered by some component of the stent in a predisposed patient carrier of non-disease-specific ANA, with an exaggerated immune response. No recurrence of ISR occurred after the introduction of steroids. Biological and intra-stent causes of ISR should be taken into careful consideration to aim for the early detection of the underlying mechanism of restenosis and to embrace the best therapeutic strategy.

  • Clara Long, Abdulrahman Al-Abdulmalek, Jonathan Lai, David G. Haegert, Stephane Isnard, Denis Cournoyer, Jean-Pierre Routy
    Views: 587 HTML: 33 PDF: 340

    Background: Autoimmune diseases are not contraindications for immune checkpoint inhibitors (ICI) therapy in patients with cancer. However, immune-related adverse events (irAEs) are frequently observed in patients receiving ICIs including dermatitis, thyroiditis, colitis, and pneumonitis. Thrombocytopenic purpura, aplasia, and haemophagocytic lymphohistiocytosis (HLH) are rarely observed during ICIs.
    Case description: We report the case of a male patient with pre-existing untreated HLA B27 and ankylosing spondylitis with gastric cancer and liver metastases. The 79-year-old man was treated with anti-HER2 trastuzumab and anti-PD-1 nivolumab. Seventeen days after the seventh cycle of treatment, he presented at the emergency department with acute fever, confusion, and hypotension. Laboratory results showed pancytopenia, and elevation of ferritin and triglyceride. No infections were detected. Although not seen in a bone marrow biopsy, clinical presentation, and absence of infection, together with an H-score of 263, indicated HLH. The patient was treated with dexamethasone for four days and discharged on a tapering dose of steroids. At the two-month follow-up, clinical presentation was normal and blood test almost normalised. At 8 months, no liver metastases were observed.
    Conclusions: In a patient with a pre-existing autoimmune condition, immunotherapy led to the development of HLH, which was controlled by glucocorticoid. Absence of the feature of haemophagocytosis in the bone marrow biopsy did not exclude the diagnosis, as HLH can occur in the spleen or in the liver. Glucocorticoid therapy did not prevent the anti-cancer effect of ICIs, and liver metastases disappeared 8 months post-HLH. This case warrants further research on the interplay between autoimmunity and ICI response, as well as ICI-induced irAEs.

  • Sofia Miranda, Patrícia Clara, Joana Rua, Miriam Cimbron, Fernando Salvador
    Views: 351 HTML: 72 PDF: 304

    Introduction: We present a clinical case of a 45-year-old man with recurrent deep vein thrombosis (DVT) and multiple hospital admissions due to severe infectious conditions. A newfound hypoalbuminemia raised the suspicion of a protein-losing condition, with an upper endoscopy revealing lesions at the D2 level compatible with coeliac sprue and HLA typing positive for both DQ-2 and DQ-8.
    Methods: A gluten free diet was started and apixaban was suspended.
    Results: No new complications were reported.
    Discussion: Multiple mechanisms are believed to be behind the association between DVT and coeliac disease. However, to this date, no consensus exists regarding the ideal duration of anticoagulation.

  • Haruka Kobayashi, Yoji Hoshina, Hidemasa Higa, Takashi Watari
    Views: 409 HTML: 92 PDF: 393

    Introduction: Guillain-Barré syndrome is an immune-mediated inflammatory polyneuritis characterised by rapidly progressive flaccid paralysis. Guillain-Barré syndrome may present with posterior reversible encephalopathy syndrome or reversible cerebral vasoconstriction syndrome in rare cases.
    Case description: A woman in her 60s with a history of follicular lymphoma presented with a one-week history of difficulty walking and thunderclap headaches. The patient was diagnosed with Guillain-Barré syndrome based on neurological examination, cerebrospinal fluid analysis and nerve conduction findings. Further diagnosis of posterior reversible encephalopathy and reversible cerebral vasoconstriction syndromes was based on imaging findings and headache history. The patient was treated with intravenous immunoglobulin and amlodipine, and symptoms improved.
    Discussion: We reviewed the literature on Guillain-Barré syndrome associated with posterior reversible encephalopathy and/or reversible cerebral vasoconstriction syndrome. The underlying pathophysiology may involve dysautonomia resulting in unstable blood pressure, and hyponatraemia causing endothelial dysfunction. The SNOOP mnemonic highlights the ‘red flags’. This SNOOP mnemonic suggests the possibility of secondary headaches that require imaging studies. In this case, the patient exhibited three SNOOP symptoms: S (history of malignancy: follicular lymphoma), O (sudden-onset headache) and O (over 50 years old).
    Conclusion: This case highlights the importance of considering coexisting central neurological disorders in patients with Guillain-Barré syndrome.

  • Alper Tuna Güven, Yusuf Ziya Demiroğlu, Nazım Emrah Koçer
    Views: 282 HTML: 58 PDF: 280

    Intravesical bacillus Calmette-Guérin (BCG) is used for urothelial carcinoma. Systemic side effects are rare and commonly include organ involvement but rarely include bone marrow. We describe a patient who had received intravesical BCG and presented shortly afterwards with constitutional symptoms. Initial work-up revealed pancytopenia and immune haemolysis. He was presumptively diagnosed with systemic BCG infection and secondary warm autoimmune haemolytic anaemia. Isoniazid, rifampin and ethambutol was started. The bone marrow biopsy revealed granulomas. Within 6 weeks of treatment, the patient's clinic and laboratory results were dramatically improved. A high level of suspicion is crucial for diagnosis and treatment.

  • Ossama Maadarani, Leila Bigdelu, Zouheir Bitar, Mohammad Alhabibi, Hosni Kabbara
    Views: 382 HTML: 61 PDF: 295

    Cardiac conduction disorder may have a wide range of aetiology and can manifest with symptomatic bradycardia and syncope. Celiac disease is a malabsorptive long-term autoimmune disorder where the small intestine is the primarily affected organ due to gluten intolerance in genetically predisposed individuals. The associations between celiac disease and cardiac pathology are uncommon. We report a case of a 50-year-old woman with a known case of celiac disease who presented with a symptomatic cardiac conduction abnormality that improved with a gluten-free diet.

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