Pemphigus vulgaris is a severe autoimmune blistering disease that occasionally develops lesions refractory to conventional systemic immunosuppressive therapy. We report the first documented case of successful treatment of therapy-resistant pemphigus vulgaris using cold atmospheric plasma. A 34-year-old woman presented with extensive oral and genital erosions that dramatically improved after initiating therapy with pulse corticosteroids in combination with cyclophosphamide. In the following weeks, she developed treatment-resistant facial erosions that persisted for 6 months despite aggressive systemic therapy consisting of intravenous immunoglobulin, azathioprine, and plasmapheresis. After 3 weekly cold atmospheric plasma treatments at 33 to 37 °C with a 2 cm torch gap for 3 min per treatment zone, the patient had remarkable clinical improvement, with an estimated > 80% epithelialization of facial erosions within 4 weeks, progressing to complete epithelialization by week 6. Disease activity assessment using the Pemphigus Disease Activity Index confirmed rapid improvement from a baseline of 14 to 0 within 6 weeks. Indirect immunofluorescence was negative throughout treatment. Sustained disease remission was achieved for 24 months. This novel application of cold atmospheric plasma is a promising therapeutic approach for recalcitrant lesions of pemphigus vulgaris.

Distal renal tubular acidosis (dRTA) is associated with autoimmune disorders, particularly Sjögren's syndrome. Hypokalemic muscle weakness due to dRTA is an uncommon presentation of Sjögren's syndrome and may mimic primary hypokalemic periodic paralysis, resulting in a diagnostic delay. We present the case of a 35-year-old woman who reported recurrent limb weakness in the presence of hypokalemia (serum potassium: 2.7 mmol/L). She had previously been diagnosed with primary hypokalemic periodic paralysis, but this diagnosis was challenged as she was found to have normal anion gap metabolic acidosis and an alkaline urine pH with significant potassium wasting. The patient reported the presence of subtle sicca symptoms only on direct questioning. Further workup revealed positive serology for Sjögren's syndrome. She was managed with potassium and sodium bicarbonate replacement and given oral steroids and azathioprine for long-term management. Following correction of electrolyte abnormalities, the patient's muscle strength improved significantly, and she regained functional mobility. Her metabolic acidosis also resolved, and she experienced no further episodes of weakness or electrolyte imbalance during 12 months of follow-up. Primary Sjögren's syndrome-associated distal RTA should be considered in patients presenting with recurrent hypokalemic paralysis, particularly in the presence of normal anion gap metabolic acidosis and alkaline urine pH. Early treatment prevents recurrent paralysis and long-term renal complications.

Hypersensitivity pneumonitis (HP) is an interstitial lung disease triggered by inhalation of a variety of environmental antigens. HP ranges from acute manifestations to progressive fibrosis and can be classified as nonfibrotic or fibrotic based on imaging and histopathological features. The pathophysiological mechanism of HP involves an exaggerated immune cell response and, in chronic stages, fibroblast activation leading to fibrosis. Diagnosis requires integrated assessment of exposure history, HRCT findings, BAL findings, and histopathological findings, as well as multidisciplinary discussion. In nonfibrotic HP patients, HRCT typically shows ground-glass opacities, centrilobular nodules, and mosaic attenuation, whereas, in fibrotic HP patients, HRCT often shows reticulation, traction bronchiectasis, and honeycombing. Typical histological features include cellular bronchiolitis, poorly formed granulomas, and interstitial fibrosis. Recent advances in imaging-including artificial intelligence-based pattern recognition-and investigation of biomarkers are improving precision and will likely contribute to the diagnostic investigation of HP. Management strategies depend on disease phenotype and severity, with antigen avoidance remaining the cornerstone of therapy. Immunomodulators such as corticosteroids, azathioprine, and mycophenolate may be used when there is evidence or suspicion of active inflammatory disease, whereas nintedanib is indicated for progressive fibrotic forms. Emerging phosphodiesterase 4B inhibitors have shown promise based on recent evidence. A greater number of comorbidities are observed in patients with fibrotic HP and are associated with worse survival, particularly pulmonary hypertension. Understanding genetic predisposition, comorbidities, and the transition from inflammation to fibrosis is essential to guide personalized treatment and improve outcomes. This review provides an update of pathophysiology, current diagnostic approaches, and therapeutic options, including emerging strategies.

Background: Myasthenia gravis is an autoimmune disorder of the neuromuscular junction characterized by fluctuating skeletal muscle weakness. Late-onset MG (onset ≥ 50 and <65 years) and very late-onset MG (VLOMG; onset ≥ 65 years) are increasingly recognized subgroups, and diagnosis in very elderly patients remains challenging because symptoms frequently overlap with age-related conditions and comorbidities. Case Presentation: An 88-year-old man with very late-onset myasthenia gravis (symptom onset at approximately age 85) was urgently referred because of a several-day history of rapidly worsening dysphagia and dysarthria, superimposed on fluctuating diplopia, dysphagia, dysarthria, and fatigable bulbar symptoms that had progressively worsened over the preceding three years. Initial diagnostic evaluation was challenging because of advanced age, previous lacunar infarctions, and multiple comorbidities, including pulmonary thromboembolism, chronic kidney disease, and permanent pacemaker implantation (which precluded brain MRI). Neurological examination and the characteristic fluctuation of symptoms raised suspicion of myasthenia gravis. Serological testing confirmed markedly elevated acetylcholine receptor antibodies, whereas MuSK antibodies were negative. Repetitive nerve stimulation was not performed given the high antibody titer and unambiguous clinical presentation. Thoracic computed tomography excluded thymoma. Treatment with pyridostigmine, azathioprine (maintenance dose kept lower than standard due to chronic kidney disease stage IIIB), and low-dose prednisone (selected due to age and comorbidity profile) resulted in early, patient-reported clinical improvement (approximately 60% in speech and swallowing) over eight weeks of follow-up; a validated severity scale (MG-ADL) showed a score of six (scoring range 0-24). Conclusions: Myasthenia gravis should remain an important differential diagnosis in very elderly patients presenting with fluctuating ocular and bulbar symptoms, even in the presence of multiple comorbidities that may obscure the diagnosis. In this patient, early recognition, antibody testing, and individualised initiation of therapy were followed by meaningful short-term improvement; a single case with eight weeks of follow-up cannot establish that such therapy prevents disease progression or myasthenic crisis, and longer follow-up and additional cases are needed.

To review current evidence on pregnancy, neonatal, and long-term outcomes in infants born to transplant recipients, with particular emphasis on the effects of maternal immunosuppressive therapy, and to present data from a Polish cohort, including breastfeeding exposure. A narrative review of the literature was conducted, focusing on maternal, neonatal, and long-term outcomes after solid organ transplantation. Registry data, cohort studies, and pharmacokinetic analyses of immunosuppressive drug transfer into breast milk were included. Findings from a Polish cohort of transplant recipients and their offspring were analyzed to complement available evidence.

A man in his 80s presented with a one-week history of jaundice, abdominal pain, low-grade fever, pruritus, and anorexia. Biochemistry showed a hepatocellular pattern of liver injury, with aspartate aminotransferase of 867 U/L and alanine aminotransferase of 702 U/L, alongside conjugated hyperbilirubinemia and a prolonged international normalized ratio. Contrast-enhanced computed tomography of the abdomen showed a bulky pancreatic head and uncinate process with mild common bile duct dilatation and no discrete mass, alongside a markedly elevated CA 19-9 of 225 U/mL, which together raised initial concern for pancreatico-biliary malignancy. A comprehensive viral hepatitis panel, including hepatitis A, B, C, and E, and thyroid function testing were unremarkable. Further workup revealed a strongly positive antinuclear antibody at high titer and an elevated total immunoglobulin G. A clinical diagnosis of autoimmune hepatitis was made using simplified international diagnostic criteria; liver biopsy was offered but declined by the patient due to his advanced age and practical constraints. Treatment with corticosteroids and azathioprine led to complete biochemical normalization over several weeks, with sustained improvement on maintenance therapy. This case demonstrates that autoimmune hepatitis can closely mimic pancreatico-biliary malignancy on cross-sectional imaging and tumor markers in an older patient, and highlights the diagnostic value of autoimmune serology and immunoglobulin G levels in avoiding unnecessary invasive oncological investigation.

Renal artery inflammation is typically observed as part of systemic vasculitis rather than as an isolated condition. Large vessel vasculitides, such as Takayasu arteritis and giant cell arteritis, as well as polyarteritis nodosa, may involve multiple vessels, including the renal artery. Isolated vasculitis of the renal artery is exceedingly rare with only eight cases reported worldwide. We describe a case of a previously healthy 53-year-old man who presented with a history of right flank pain which was attributed to renal artery vasculitis. Extensive radiologic studies revealed no evidence of inflammation in other vessels and laboratory evaluation for an underlying infectious, neoplastic or autoimmune cause was negative. Treatment with methylprednisolone, followed by azathioprine as a steroid-sparing agent, led to complete remission of the condition.

Thiopurine drugs are used for the treatment of a wide variety of diseases ranging from autoimmune diseases to various leukemia types. As efficacy of thiopurine drugs is significantly reduced by Thiopurine S-methyltransferase (TPMT)-mediated S-methylation, accurate assessment of individual TPMT-activity levels is crucial for optimizing thiopurine drug dosage regimens for each patient. Given its multifaceted regulation, identifying occupational exposures impacting TPMT enzymatic activity is crucial. The influence of lead(Pb[2 +]) on the enzymatic activity of TPMT was initially assessed in vitro, by spiking human blood having normal TPMT levels with increasing levels of Pb[2+]. The effect of occupational Pb[2+]-exposure on TPMT activity was further investigated by comparing blood TPMT activity levels of industrial workers prone to occupational Pb[2+]-exposure. Putative Pb[2+]-docking sites on TPMT enzyme were assessed through in silico analysis via MIB2 and CB-Dock2 servers. In vitro analysis indicated an inverse correlation between blood Pb[2+]-levels and TPMT-activity, which was further supported by in vivo analyses, providing evidence for Pb[2+]-mediated TPMT-inactivation. In silico Pb[2+]-docking analyses suggest two putative regions for Pb[2+]-mediated competitive TPMT-inhibition, four regions for direct/indirect inhibition, and four regions for allosteric inhibition. Further kinetic studies suggest that Pb[2+] exerts its inhibitory effect through competition with SAM for the active site. The observed inverse correlation between blood Pb[2+] and TPMT levels emphasizes the clinical importance of individualizing thiopurine drug dosage, especially for patients exposed to high levels of Pb[2+]. In vitro studies involving site-directed mutagenesis and analysis of patients with Pb[2+]-binding site mutations will validate the precise Pb[2+]-binding sites involved in TPMT-inhibition.

Bilateral basal ganglia involvement is an uncommon but treatable manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE) that is rarely described in pediatric patients and is frequently omitted from the standard differential diagnosis for bilateral basal ganglia lesions in childhood. We report an adolescent girl with a recent diagnosis of systemic lupus erythematosus who presented with acute neuropsychiatric deterioration, including altered mental status and seizures. Brain magnetic resonance imaging (MRI) demonstrated symmetrical T2-weighted/fluid-attenuated inversion recovery (T2/FLAIR) hyperintensity of the bilateral anterior basal ganglia without diffusion restriction, consistent with vasogenic edema. Concomitant findings included a left cerebellar subacute cortical infarct and diffuse leptomeningeal enhancement, a constellation postulated to reflect diffuse systemic lupus erythematosus-related endothelial dysfunction and blood-brain barrier disruption. Infectious and metabolic etiologies were excluded. The patient was managed with pulse methylprednisolone and azathioprine without significant improvement. Subsequent initiation of plasmapheresis resulted in clinical improvement, supporting a humorally mediated mechanism of injury. This case highlights the importance of including NPSLE in the differential diagnosis of symmetrical basal ganglia lesions in children, particularly when systemic autoimmune features are present, to ensure timely escalation of therapy.