A woman with rheumatoid arthritis, Sjögren's Syndrome, leg ulcer, and significant weight loss

Kerr, Gail S.; Aggarwal, Anita; McDonald-Pinkett, Shelly

doi:10.1002/acr.21628

Cited by 4 publications

(4 citation statements)

References 48 publications

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“…The co-occurrence of primary Sjögren's syndrome with pyoderma gangrenosum is an infrequent phenomenon documented in the literature. Among the seven previously reported cases (6)(7)(8)(9)(10)(11)(12), five were women, and the majority of cases occurred between the fourth and sixth decades of life. Except for a 25-year-old female patient (11), all cases were diagnosed with pSS before the occurrence of PG.…”

Section: Discussionmentioning

confidence: 97%

“…Although the first findings are mostly sicca symptoms, the heterogenous nature of SjS as a multisystemic disease may affect gastrointestinal, pulmonary, neurologic, articular, and cutaneous organs (1,2). Rare examples of this multisystemic involvement include case reports in the literature declaring first clinical presentations with cerebellar degeneration, pulmonary amyloidosis, membranous nephropathy, or pyoderma gangrenosum (PG) (3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

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A Rare Manifestation Of Primary Sjogren’s Syndrome With Pyoderma Gangrenosum : Case Report

Nalbant

2023

Med Sci Discov

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Objective: Sjögren’s Syndrome (SjS) is a systemic autoimmune disease typically presenting with sicca symptoms. However, a limited number of case reports in the literature have described rare initial presentations with pyoderma gangrenosum (PG). This report aims to underscore this infrequent association between PG and SjS by presenting another illustrative case. Case: This case report elucidates the diagnosis and treatment of primary Sjögren’s syndrome (pSS), focusing on the ulcerated lesion that manifested on the leg of a 54-year-old female patient with no underlying health conditions. The findings are discussed in the context of the existing literature. Discussion: SjS is a multisystemic autoimmune disease characterized by lymphocyte infiltration and damage to tissues, especially exocrine glands. Although sicca symptoms are typically the initial clinical findings, PG can rarely manifest as the primary symptom, presenting as an ulcerative skin lesion predominantly located on the lower extremities. There is currently no standard treatment recommendation in the existing guidelines for this rare condition. Conclusion: The co-occurrence of PG and SjS is uncommon, yet it may serve as a warning for associated systemic diseases, such as SjS. Treatment may involve a combination of corticosteroids and disease-modifying antirheumatic drugs (DMARDs).

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Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

A Rare Manifestation Of Primary Sjogren’s Syndrome With Pyoderma Gangrenosum : Case Report

Nalbant

2023

Med Sci Discov

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“…[5][6][7] Furthermore, PG may be the first clinical sign in patients with rheumatoid arthritis and SjS who having resistant leg ulcers on their lower extremities. 8,9 Although the etiopathogenesis of both diseases is quite different and poorly understood, a common unknown triggering factor may lead to dysregulation in the autoimmun system involving the activation of two distinct inflammatory pathways in the same patient. Besides, it might develop a cutaneous vasculitis characterized by palpable purpura, especially in some patients with SjS in which detected the hypergammaglobulinemia or cryoglobulinemia.…”

Section: Discussionmentioning

confidence: 99%

Ulcerative Type Pyoderma Gangrenosum in Primary Sjogren's Syndrome

Gündoğdu¹,

Çiçek²,

Koca³

2018

Turkiye Klinikleri J Case Rep

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yoderma gangrenosum (PG) is a rare neutrophilic dermatosis which presents as an inflammatory and ulcerative disorder of the skin. Although the etiology of PG is unknown, the impaired neutrophil chemotaxis plays an important role in the pathophysiology of PG. 1,2 Sjogren's syndrome (SjS) is a systemic, inflammatory disease characterized by lymphocytic infiltration of the exocrine organs, 3 Dryness of the skin, cutaneous vasculitis and Raynaud's phenomenon can occur in SjS. However, PG is not a common skin finding in SjS. In this case report, a rare association of PG and SjS has been revealed based on a review of the literature. CASE REPORTA 63-year-old female patient with SjS was admitted to rheumatology department because of an ulcer in her left leg (Figure 1). The patient had applied to the rheumatology polyclinic with dry mouth, dry eyes, diffuse joint pain four years ago. She patient's laboratory tests had revealed an increase in acute-phase response with the positivity for antinuclear antibody-indirect immunofluorescence (ANA-IFA) test (1/320 titre, speckled pattern) and anti-Sjogren's syndrome antibody (anti-SSA) (3+, ELISA) test. Schirmer's test had been bilaterally detected less than 5 mm. The minor salivary gland Turkiye Klinikleri J Case

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“…Furthermore, because of some shortcomings in the current diagnostic criteria, one must thoroughly consider other disorders in the differential diagnosis. In our case of unexplained generalized lymphadenopathy, fatigue, and night sweats, the differential diagnosis included various infections (EBV, CMV, toxoplasmosis, HIV, and mycobacterium), lymphoma/leukemia, metastatic neoplasia, systemic lupus erythematous, Castleman's disease, autoimmune lymphoproliferative syndrome, and Sjögren's syndrome [ 8 ]. However, with a thorough evaluation to exclude alternative diagnoses, in the setting of our patient's elevated serum IgG level and significant staining for IgG4, her diagnosis was established.…”

Section: Diagnosismentioning

confidence: 99%

Persistent Lymphadenopathy due to IgG4-Related Disease

Smith

Carroll

2012

Case Reports in Immunology

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A 28-year-old healthy female presented to her primary care physician with lymphadenopathy, fatigue, malaise, and night sweats. Symptoms persisted despite conservative treatment and eventually the patient underwent multiple lymph node resections and a bone marrow biopsy before a diagnosis of IgG4-related disease (IgG4-RD) was made. IgG4-RD is a relatively new disorder first histopathologically recognized within the last decade. As the disease can affect a single organ or multiple organs, symptoms can vary greatly among patients. With symptoms ranging from mild, such as lower extremity edema, to severe, such as spinal cord compression, IgG4-RD must be considered in appropriate patients. Diagnostic criteria have been proposed based on organ involvement, serum IgG4 levels, and histopathological criteria. Diagnosis can be difficult to make with many studies suggesting different values for diagnostic criteria, such as the level of tissue IgG4+/IgG+ cell ratio to delineate IgG4-RD. Treatment consists of high dose glucocorticoids as a first line therapy with some patients choosing instead to simply undergo observation. This case illustrates the difficulty in diagnosis and the need for increased awareness among medical professionals.

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A woman with rheumatoid arthritis, Sjögren's Syndrome, leg ulcer, and significant weight loss

Cited by 4 publications

References 48 publications

A Rare Manifestation Of Primary Sjogren’s Syndrome With Pyoderma Gangrenosum : Case Report

A Rare Manifestation Of Primary Sjogren’s Syndrome With Pyoderma Gangrenosum : Case Report

Ulcerative Type Pyoderma Gangrenosum in Primary Sjogren's Syndrome

Persistent Lymphadenopathy due to IgG4-Related Disease

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