Immune System Disorders

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• A rare post-infectious autoimmune manifestation of COVID-19 Fatih Kaya, Tarek Alsafdi, Manar Hussam Al-Suleh Views: 22 PDF: 12 HTML: 2

  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.

• Autoimmune myelofibrosis revealing a systemic lupus erythematosus Fatimaezzahra Bensalek, Hajar Joulal, Jaouad Yousfi, Mouna Zahlane, Leila Benjilali, Lamiaa Essaadouni Views: 23 HTML: 3 PDF: 10

  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.

• Recurrent intra-stent coronary restenosis in a carrier of non-disease-specific antinuclear antibodies Benedetta Tomberli, Stefano Fumagalli, Tiziana Cristina Minopoli, Silvia Menale, Valentina Scheggi, Niccolò Marchionni Views: 73 HTML: 7 PDF: 55

  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.

• Haemophagocytic lymphohistiocytosis following the anti-PD-1 nivolumab in a patient with gastric cancer and ankylosing spondylitis Clara Long, Abdulrahman Al-Abdulmalek, Jonathan Lai, David G. Haegert, Stephane Isnard, Denis Cournoyer, Jean-Pierre Routy Views: 302 HTML: 20 PDF: 176

  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.

• Deep vein thrombosis revealing a "silent" coeliac disease Sofia Miranda, Patrícia Clara, Joana Rua, Miriam Cimbron, Fernando Salvador Views: 321 HTML: 61 PDF: 272

  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.

• Guillain-Barré syndrome complicated by posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome Haruka Kobayashi, Yoji Hoshina, Hidemasa Higa, Takashi Watari Views: 383 HTML: 85 PDF: 361

  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.

• Systemic bacillus Calmette-Guérin (BCG) infection and accompanying warm autoimmune haemolytic anaemia following intravesical BCG immunotherapy Alper Tuna Güven, Yusuf Ziya Demiroğlu, Nazım Emrah Koçer Views: 260 HTML: 52 PDF: 253

  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.

• Spontaneous recovery of isolated advanced heart block in patient with celiac disease by starting a strict gluten free diet: a case report and review of the literature Ossama Maadarani, Leila Bigdelu, Zouheir Bitar, Mohammad Alhabibi, Hosni Kabbara Views: 357 HTML: 60 PDF: 271

  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.

• Genital ulcers associated with systemic lupus erythematosus – what are the possible causes? A Case Report Maysoun Kudsi , Tasneem Drie , Ghina Haidar, Safaa Al-Sayed, Enas Roumieh Views: 539 PDF: 294 HTML: 88

  Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease that affects many organs. In this report, we discuss the case of a patient with SLE who presented to an out-hospital clinic, complaining of fever and pain with genital ulcers. Negative evaluations for other causes of genital ulcers, indicated these ulcers as related to SLE. This case highlights the importance of including SLE ulcers in the differential diagnosis of genital ulcers.

   

• Immune reconstitution inflammatory syndrome in AIDS patient after successful induction of virological suppression with cabotegravir/rilpivirine Rami Al-Handola, Justine Chinnappan, Mohammad Bakeer, Danielle Osterholzer Views: 403 HTML: 45 PDF: 364

  Long-acting (LA) cabotegravir/rilpivirine (CAB/RPV) is a complete regimen for the management of human immunodeficiency virus type 1 (HIV-1) infection to replace their oral antiretroviral therapy (ART) when they have been virologically suppressed. We present a case of successful achievement of undetectable HIV RNA viral load levels in an acquired immunodeficiency syndrome (AIDS) patient with long-standing virologic failure within two months of CAB/RPV LA initiation. This was later complicated by immune reconstitution inflammatory syndrome (IRIS) due to Mycobacterium avium-intracellulare (MAI) infection and hepatitis B virus (HBV) reactivation.