Pham, Michael M

ABSTRACT Sjögren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocytic infiltration and destruction of exocrine glands, commonly involving the salivary and lacrimal glands, leading to dry mouth and eyes. While SS primarily affects the upper gastrointestinal tract, this case presents a rare small intestine involvement. This patient was diagnosed with SS in 2018 with positive antinuclear antibodies (ANA), rheumatoid factor (RF), SSA antibodies, and salivary lip gland biopsy. By 2021, she developed unusual cyclic episodes of nausea, vomiting, and poor oral intake, resulting in significant weight loss and recurrent hospitalizations. She had frequent hospitalizations due to her recurrent symptoms. Evaluations included esophagogastroduodenoscopy (EGD) and colonoscopy, but pathology was nondiagnostic. Several abdominal and pelvic computed tomography (CT) scans demonstrated bladder edema, pancolitis, and terminal ileitis with mucosal hyperenhancement of the bowel and moderate ascites, likely inflammatory. Her gastric emptying study showed hypermotility. Multiple teams, including Rheumatology, Allergy/Immunology, Gastroenterology, Hematology, and Surgery, were involved. C1 esterase antigen was normal, ruling out hereditary angioedema. PET‐CT scan did not show lymphadenopathy or significant inflammation. Full biopsy for definitive diagnosis was deferred due to concerns of decreased healing with an edematous intestine. She was trialed on Icatibant without benefit. Differentials included lupus peritonitis, protein‐losing enteropathy, and SS intestinal lymphoma. She was discharged on a trial of empiric high‐dose steroids with Rheumatology follow‐up. Symptoms recurred with steroid tapering. Her complement levels decreased with flares and increased with treatment. Rituximab was added, leading to steady clinical improvement and reduced hospitalizations. Bowel involvement has been predominantly described in lupus, often referred to as lupus enteritis or bowel vasculitis. To our knowledge, this represents the first reported case of Sjögren's syndrome. Bowel biopsies are not typically subjected to routine immunohistochemical staining. Consequently, standard testing may be limited in detecting autoimmune processes. This case highlights the diagnostic challenges of SS‐related gastrointestinal manifestations and underscores the importance of considering autoimmune enteropathy, particularly in patients who respond to immunosuppressive therapy.

Abstract Background: Group G streptococcal species can represent normal human flora as asymptomatic colonization. In the setting of infection, group G streptococcus can invade multiple structures and present broadly including bacteremia, toxic shock syndrome, endocarditis, septic arthritis, osteomyelitis, necrotizing skin infections, pharyngitis, and others. Severe cases tend to be more common in patients of increasing age or with chronic illnesses. Here we report a case of disseminated group G streptococcus mimicking systemic vasculitis. Case Presentation: A 41-year-old white male was hospitalized with a five-day history of fevers, chills, painful bilateral leg rash, generalized arthralgias, and right eye redness, pain and severe vision loss. Medical history was significant for minimal change disease treated with prednisone and cyclosporine. On admission, he was noted to be cushingoid with edematous erythema at the left hand, and bilateral lower limb rash. The right eye was inflamed with hypopyon. Laboratory studies demonstrated mild leukocytosis, modest thrombocytopenia, hyponatremia, hyperglycemia, with elevated CRP and creatinine. Urinalysis was positive for proteinuria, microscopic hematuria, and pyuria. CT chest demonstrated right upper lobe consolidation. There was concern for systemic vasculitis and cellulitis of the left hand. Intravenous methylprednisolone and empiric antibiotics were initiated. Ophthalmology was consulted for right uveitis and aspirated fluid from the anterior chamber. Dermatology biopsied the lower limb rash and identified leukocytoclastic vasculitis. Pulmonology performed bronchoscopy and bronchoalveolar lavage of the right upper lobe. Prior renal pathology was re-reviewed. Rheumatology was consulted due to suspicions for systemic vasculitis. Lancefield group G streptococcus was ultimately cultured from multiple blood samples, bronchoalveolar lavage fluid and right eye aspirate. There was no evidence of endocarditis on imaging. It was concluded that the source of the bacteremia and disseminated infection was most likely the left-hand cellulitis. Conclusions: To our knowledge, we present the first case of disseminated Group G streptococcal infection presenting with the constellation of bacteremia, endophthalmitis, focal mass-like lung consolidation, and leukocytoclastic vasculitis of lower extremities. The multi-organ distribution mimicked a systemic small vessel vasculitis. This case highlights the importance of infectious etiologies in the differential diagnosis of vasculitis – especially in the setting of infectious risk factors.