Pyoderma gangrenosum (PG) is a rare, chronic neutrophilic dermatosis characterized by rapidly progressive, painful ulcers. It is frequently associated with systemic inflammatory conditions such as inflammatory bowel disease. The pathogenesis remains incompletely understood, and treatment of refractory cases poses a significant challenge. We present a 26-year-old woman diagnosed with PG, initially managed with systemic corticosteroids and ciclosporin. During follow-up, she developed gastrointestinal symptoms and was subsequently diagnosed with Crohn disease (CD). Despite treatment with azathioprine, infliximab and, later, ustekinumab, the patient experienced repeated flares of both PG and CD. Upon recurrence of PG lesions and CD exacerbation under ustekinumab therapy, systemic corticosteroids were reintroduced, and subcutaneous guselkumab, an interleukin (IL)-23 p19 inhibitor, was initiated. A rapid and sustained clinical response was observed following the second dose of guselkumab, with complete ulcer healing and tapering off corticosteroids. The patient has remained in complete remission from both PG and CD for over 2 years under guselkumab monotherapy. This case adds to the growing body of evidence supporting the use of IL-23 inhibitors, particularly guselkumab, in recalcitrant PG associated with systemic inflammatory comorbidities. Guselkumab may offer a promising therapeutic alternative in the management of treatment-resistant PG, especially in patients with coexisting CD.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by rapidly progressive painful ulcers, and increasing evidence supports a role for the interleukin-36 pathway in its pathogenesis. We report the case of a 59-year-old woman with idiopathic refractory PG that evolved over 8 years. The patient had an insufficient response to prolonged systemic corticosteroids and azathioprine, with persistent steroid dependency. Spesolimab was initiated as a single 900-mg intravenous infusion in combination with low-dose prednisone (10 mg daily). This led to pain reduction, re-epithelialization and progressive ulcer healing, estimated at 30% after 3 months, 50% after 6 months and 80% after 8 months. No major adverse events were observed, apart from an erysipelas-like reaction around the ulcers 3 months later. Our review of published cases suggests that spesolimab may induce rapid and sustained improvement in refractory PG, although responses appear more heterogeneous in patients with underlying myeloid disorders. Overall, spesolimab appears to be a promising and well-tolerated therapeutic option for refractory PG, but further controlled studies are needed to better define its efficacy and safety, and place in the treatment algorithm.

Background Acute severe ulcerative colitis (ASUC) affects up to 25% of patients with ulcerative colitis. Despite intravenous (IV) corticosteroids as first-line therapy, 30-40% require rescue treatment and up to 40% undergo colectomy within one year. Upadacitinib (UPA) has shown ability to induce rapid response in moderate-severe UC, but real-world data in ASUC remain limited. We aimed to describe outcomes of UPA initiation for ASUC across Mayo Clinic sites. Methods We conducted a descriptive study of 23 adults hospitalized with ASUC between 2022-2025 at Mayo Clinic. Patients with their first ASUC admission who were started on UPA were included. All patients received IV methylprednisolone (40-60 mg/day) within 24-48 hours of admission, followed by UPA initiation (inpatient or shortly after discharge). Clinical, laboratory, and treatment data were extracted from electronic medical records. Outcomes included colectomy during admission and colectomy within one year. Descriptive statistics were done using Microsoft Excel. Results Twenty-three patients received UPA (median age 35 years; 88% pancolitis; 4% PSC). Out of the 23, 18 were started on UPA during the ASUC admission. Before admission, 10 patients (43%) were receiving corticosteroids, three (13%) were on mesalamine, one (4%) on azathioprine, and 12 (52%) were on biologic therapy; four patients (17%) were already on lower dose UPA maintenance. The median number of prior advanced therapies was two (range 1-4). Admission labs showed a mean CRP of 56.3 mg/L (SD 44.9) and fecal calprotectin of 2521.9 µg/g (SD 783.9). Patients received IV corticosteroids for a mean of 3.7 days (SD 1.1), and UPA was initiated at a median of hospital day 3 (IQR 4). 2/18 patients (11.1%), who were started on UPA inpatient, required colectomy during admission. Of the 23 patients who received UPA during hospitalization or shortly after discharge, one year colectomy rate was 4/23 (17%). Conclusions In this cohort, initiation of UPA during hospitalization or shortly after discharge for ASUC was feasible and was associated with lower colectomy rates compared with published outcomes for patients treated with infliximab or cyclosporine, both during hospitalization (11.1% vs. 21% vs. 25%) and at one year (17% vs. 35% vs. 45%). These findings suggest that UPA may serve as an effective rescue option for hospitalized ASUC patients, particularly when rapid control of inflammation is needed. Larger prospective studies are warranted to confirm these observations and to define optimal timing, patient selection, and long-term safety in the ASUC setting.

ObjectiveTo characterize the burden of herpes zoster (HZ) in systemic lupus erythematosus (SLE) and to examine clinical correlates of HZ and patients' attitudes toward the recombinant zoster vaccine (RZV).MethodsWe conducted a single-center retrospective cohort study combined with a cross-sectional survey among consecutive adult outpatients with SLE between December 2024 and June 2025. HZ episodes after SLE diagnosis were ascertained via structured interviews. Incidence rates were calculated as the number of HZ episodes per 1000 person-years from SLE diagnosis to enrollment. Participants were classified into three groups according to the number of HZ episodes: HZ[0], HZ[1], and HZ[≥2] groups. Factors associated with HZ development were assessed using multivariable logistic regression. Patients were asked about willingness to receive RZV ("yes", "no", or "not sure") and reasons for the response.ResultsAmong 170 patients with SLE, 73 (43%) reported at least one HZ episode after SLE diagnosis, and 29 (40% of those with HZ) reported recurrent HZ. During 3138 person-years of follow-up, at least 102 HZ episodes were reported, yielding a minimum incidence of 32.5 per 1000 person-years. In multivariable analysis, ever use of azathioprine (OR 2.74, 95% CI 1.03-7.29) and renal dysfunction (OR 2.59, 95% CI 1.04-6.48) were associated with HZ development. Attitude data were available for 159 patients: 31 (20%) were willing to receive RZV, 47 (30%) were unwilling, and 81 (50%) were unsure.ConclusionsHZ was common and frequently recurrent in this Japanese SLE cohort. Ever use of azathioprine and renal dysfunction were associated with HZ development, while willingness to receive RZV was low. Risk-stratified clinician counselling and shared decision-making may improve acceptance of HZ prevention strategies in high-risk patients with SLE.

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.