Immune System Disorders

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• Deep vein thrombosis revealing a "silent" coeliac disease Sofia Miranda, Patrícia Clara, Joana Rua, Miriam Cimbron, Fernando Salvador Views: 368 HTML: 79 PDF: 319

  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: 434 HTML: 92 PDF: 413

  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: 291 HTML: 59 PDF: 292

  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: 395 HTML: 61 PDF: 305

  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: 623 PDF: 329 HTML: 98

  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: 453 HTML: 52 PDF: 417

  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.

• Anti-N-methyl-D-aspartate receptor encephalitis, a diagnosis not to miss Tiago Valente, Gisela Gonçalves, Valter Duarte, Gorete Jesus Views: 495 PDF: 387 HTML: 134

  Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis is a rare autoimmune disease, characterized by the presence of neuropsychiatric symptoms. It is sometimes mistaken for a psychiatric disorder and other times not considered in the differential diagnosis of an encephalitic process. Correct identification of this disease and prompt treatment are key for optimal recovery, which might take weeks to months. Many patients manifest severe symptoms, with depressed level of consciousness, breathing dysfunction and dysautonomia requiring admission to the Intensive Care Unit (ICU). We report the case a young male patient with anti-NMDA encephalitis who presented typical neuropsychiatric symptoms. Despite being diagnosed and treated in a timely manner, he did not respond well to first-line immunotherapy and was admitted to the ICU with neurological, respiratory, and cardiovascular dysfunction. This resulted in prolonged hospital admission and many infectious complications. Despite the severity of the disease, the patient managed to recover in the months following discharge from hospital.

• Wells syndrome with generalized oedema Magdalini Manti, Efthymia Pappa, Vasiliki Arkouli, Leonidas Marinos, Constantinos Christopoulos Views: 404 HTML: 62 PDF: 333

  Wells syndrome or eosinophilic cellulitis is a rare and relapsing skin disease which lacks systemic involvement. A skin biopsy is needed to establish a diagnosis. Several precipitating factors have been proposed but no proven causative link has been found. On the other hand, Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), an auto-immune disease, is associated with multiorgan, including cutaneous manifestations. We report a case with overlapping features of Wells and Churg-Strauss syndrome, suggesting that these syndromes could be part of the same nosological entity.

• RS3PE syndrome with subsequent PMR caused by long-term DPP-4 inhibitor use Yuichi Takahashi, Gautam Deshpande, Yoshinori Kanai, Kwang-Seok Yang, Toshio Naito Views: 554 HTML: 66 PDF: 477

  Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome has been reported in patients treated with dipeptidyl peptidase-4 inhibitors (DPP-4i). We experienced a case of RS3PE syndrome in a 73-year-old man with a history of type 2 diabetes, who developed RS3PE as a side effect of vildagliptin. Further to this, the patient developed polymyalgia rheumatica (PMR), the first such case associated with long-term DPP-4i use.

• Disseminated necrotic suppurative ulcers as a manifestation of cocaine abuse Álex García Tellado, Carmen Lasa Teja, María Rodríguez Vidriales, Mercedes De la Fuente Vázquez, Patricia Marín Oliván, Francisco Arnaiz Las Revillas Almajano Views: 352 HTML: 64 PDF: 339

  Introduction/Aims/Background: Pyoderma gangrenosum is an immune-mediated illness that can be caused by several affections, such as inflammatory bowel disease, rheumatoid arthritis, and drug use. We present a rare case of pyoderma gangrenosum induced by levamisole-adulterated cocaine. There have been few cases of this disease reported in the world. Levamisole is an anthelmintic drug used to adulterate cocaine to boost its effect. It also has immune-modulating effects causing, among others, vasculitis and dermatological problems.
  Materials and Methods: Clinical case of a 46-year-old man admitted to the hospital University Marqués de Valdecilla in Santander, Spain, in August 2022. We diagnosed pyoderma gangrenosum based on clinical, analytical, and histological parameters.
  Results: We report a case of pyoderma gangrenosum induced by consumption of levamisole-adulterated cocaine.
  Discussion: This patient suffered from a rare and extensive immune-mediated affection with characteristic primary lesions in the form of suppurative ulcers that responded to immunosuppressive treatment. Behind pyoderma gangrenosum there may be underlying conditions such as inflammatory bowel disease, or pyoderma gangrenosum may be secondary to identifiable causes such as cocaine use as in this patient.