Hereditary gelsolin amyloidosis is an adult onset autosomal dominant disease with neurologic, ophthalmologic and dermatologic features that may be mistaken for Sjögren syndrome. We describe a case of a 68 year old female presenting with facial numbness and asymmetry, dry eyes, dry mouth and fatigue, originally diagnosed as Sjögren syndrome and treated with hydroxychloroquine. Due to her insidious progression of facial weakness with associated proximal muscle weakness she underwent a muscle biopsy, which demonstrated features of hydroxychloroquine induced myopathy and amyloid deposition. This subsequently led to targeted genetic testing, revealing an autosomal dominant c.640G > A pathogenic variant of the gelsolin gene. Therefore, this is a unique case of complex muscle pathology with features of a rare hereditary systemic amyloidosis an uncommon drug- induced myopathy.

UNASSIGNED: Sjögren\'s syndrome is an autoimmune disease affecting exocrine glands and other organs. At the onset of the disease and during its course, it most commonly manifests as neuropsychiatric symptoms, most frequently, depression and anxiety. However, the association with bipolar disorder seems rare and not frequently documented.
UNASSIGNED: Our case report presents a 43-year-old male complaining from neuropsychiatric symptoms including but not limited to periods of elevated mood, increased energy, headaches. Examination revealed xerophthalmia and xerostomia, thus Sjogren\'s Syndrome was diagnosed. A psychiatric consultation further diagnosed the patient with Bipolar Disorder. Successful treatment consisted of Carbamazepine and Azathioprine.
UNASSIGNED: People with immunological diseases like Sjogren\'s Syndrome have a higher chance of developing Bipolar Disorder with an unclear etiology. Clinical symptoms, MRI findings, and cerebral fluid analysis can lead to a proper diagnosis. Treatments include usual treatment for Sjogren\'s such as corticosteroids and immunosuppressants, in the addition of mood stabilizers for Bipolar.
UNASSIGNED: Psychiatry assessments should be done systematically in patients with autoimmune diseases to avoid delays in diagnosis and to make better treatment decisions.

To strengthen the understanding of rheumatic diseases (RDs) as the most common underlying conditions associated with acquired hemophilia (AH), a potentially fatal bleeding condition due to the development of autoantibodies or inhibitors to coagulation factor VIII, and rarely to factor IX, here we presented two cases of RDs associated AH to elucidate the disease progression, treatment, and prognosis. The presented 2 cases showed good responses to glucocorticoid (GC) and immunosuppressive agents. And then, a case-based systematic review was conducted to better understand the clinically practiced diagnosis and treatment of RDs associated AH. A total of 14 articles were included in the final literature review. All the identified 14 patients with underlying RDs and AH presented with bleeding symptoms, increased APTT, decreased FVIII activity, and positive FVIII inhibitors. Twelve of the 14 patients (85.7%) started an eradication of autoantibodies treatment with GC and immunosuppressive agents. Among which six patients achieved partial or complete remission, and four patients (28.6%) switched to Rituximab and responded well. Nine of the 14 patients received hemostasis therapy, including recombinant human FVIIa (rFVIIa). Two patients (14.3%) died due to mass bleeding and key organ failure. AH should be highly suspected in patients with RDs presenting spontaneous mucocutaneous or internal bleeding and an isolated prolonged APTT. Given the high morbidity of AH, it is important to facilitate efficient and proper management.

Sjogren\'s syndrome (SS) is a systemic autoimmune disease marked by lymphocyte infiltration of the exocrine glands and a variety of systemic symptoms. The wide range of prevalence reported in different studies is due to the fact that SS respiratory symptoms are polymorphic and vary in severity. Some 9%-20% of patients with SS have clinically severe lung impairment. Pleural effusion in SS has an etiology that is unknown. It is thought to be caused by CD4+ T cells secreting cytokines that cause B lymphocytes to generate autoantibodies. High beta-2-microglobulin, which is secreted by lymphocytic tissue particularly in pulmonary SS, is another sign of lymphoproliferation in lung tissue. Our patient had recurrent pleural effusion due to lymphoproliferation in the lung as a result of SS.

OBJECTIVE: To present a case of Sjogren syndrome with pulmonary manifestations in an adult female and discuss its assessment and management.
METHODS: Case Report.
METHODS: Tertiary care hospital.
METHODS: One.
RESULTS: A 50 yrs female admitted with complaints of dryness of eyes with decreased salivation causing difficulty in swallowing since last 3 years, with persistent dry cough since 10-15 days and progressive dyspnea since 4-5 days. Anti-nuclear antibody (ANA) profile revealed Anti- Ro/SS-A and Anti- La/SS-B Positive. Also, sub-lingual excisional biopsy was done which was consistent with findings of Sjogren\'s syndrome. Patient showed significant improvement after starting oral glucocorticoids, systemic anti inflammatory agents (Tab. HCQS), artificial tear drops, oral iron supplements and other supportive treatment.
CONCLUSIONS: Sjögren syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function and associated with lymphocytic infiltration of exocrine glands, and can affect extraglandular organ systems including the skin, lung, heart, kidney, neural, and hematopoietic systems. We present a case of Sjogren syndrome in an adult female presenting with xerostomia and dyspnea and was diagnosed upon detection of anti-Ro and anti-La antibodies and confirmed by histopathological examination of lip biopsy. Patient was started on oral steroids and other supportive treatment, General condition improved significantly and is doing very well on regular follow-up.

10% of rheumatoid arthritis (RA) cases are associated to so-called secondary Sjögren\'s syndrome (SS). These RA cases have higher DAS, fewer remissions. Is this linked to a poor response to DMARDs (disease-modifying anti-rheumatic drugs)? No study has addressed this question to date. Does the association between secondary Sjögren\'s syndrome (SS) and rheumatoid arthritis (RA) affect the therapeutic response to DMARDs and long-term prognosis? We conducted a retrospective case-control study: 39 RA associated with SS was (anti-SSA antibodies and/or Chisolm stage III or IV) were compared to 39 isolated cases of erosive RA matched by age, duration of progression and gender. The DAS CRP was higher in the RA + SS group in patients with disease progression of 16 years: 2.6 (1.5-4.5) compared to the RA group: 1.6 (1.3-2.8) (p = 0.0001) while fewer patients were in remission: 61 vs. 92% (p = 0.002). A higher number of B DMARDs have been prescribed: RA + SS = 3.04 (1-7); RA = 1.7 (1-5) (p = 0.004). Anti-TNFs are less effective when RA is associated with SS: 30 vs. 70%. Conversely, Rituximab is more effective when RA is associated with SS: 80 vs. 30%. Erosive RA-related SS exacerbates the clinical course of the condition: higher DAS, fewer remissions. This is linked to reduced treatment efficacy: higher number of DMARDs prescribed, reduced efficacy of anti-TNF drugs. RA-related SS could modify sensitivity to biotherapies: lower percentage of remissions and resistance to anti-TNF drugs.

We describe a case of Sjögren\'s syndrome-associated trigeminal neuropathy with mandibular nerve enhancement at the foramen ovale and Gasserian ganglion (Meckel\'s cave) in a patient with a prior history of breast cancer. We also explore the differential diagnosis of trigeminal neuropathy associated with mandibular nerve involvement at the foramen ovale and exclude other diseases such as Sjögren\'s syndrome or perineurial invasion as a result of various carcinomas. We emphasize the importance of an investigative triad of searching for a local head-and-neck malignancy that may metastasize by perineural spread or invasion, excluding a distant malignancy or paraneoplastic phenomenon and ruling out an autoimmune etiology such as Sjögren\'s syndrome. In the process, we briefly outline the basic immunopathologic processes.

Pulmonary involvement in Sjogren syndrome (SS) could manifest as cystic lung disease (CLD). CLD in SS includes lymphocytic interstitial pneumonia (LIP) and pulmonary amyloidosis. Differential diagnosis usually requires surgical lung biopsy, whereas CT-guided percutaneous fine needle aspiration biopsy (CT-FNAB) has not yet explored. We describe the case of a 63-year-old never smoker Caucasian female with a SS diagnosis who displayed a newly detected diffuse CLD at high-resolution computed tomography, though totally asymptomatic. Given the favorable location of one big lesion at the superior left lobe, a CT-FNAB was proposed instead of a more invasive SLB. At histology examination a diagnosis of pulmonary nodular AL kappa amyloidosis in the context of SS was established. In conclusion, CT-FNAB might represent an alternative and less invasive diagnostic procedure than SLB in the differential diagnosis of CLD, even if further research is needed. Moreover, this case presents an unusual association between SS and pulmonary nodular AL kappa amyloidosis, with pulmonary nodules and cysts without systemic manifestations.

Sjogren\'s syndrome (SS) is a chronic autoimmune disorder that causes the inflammation of the lacrimal and salivary glands, resulting in dryness of the eyes and mouth. In addition, fatigue and musculoskeletal pain, often described as aching, are very common. Treatment directed towards alleviating the fatigue and pain associated with SS is currently very limited. In March of 2019, the first peer reviewed case report showing clinical improvement using low-dose naltrexone (LDN) in a patient with suspected SS was published in Cureus. This report describes two additional patients with SS whose conditions responded favorably to a treatment with LDN therapy. The first case is a 24-year-old female with documented SS. Her diagnosis was based on a history of dry eyes, dry mouth, joint pain, fatigue, and headache. In addition, she had very high measures of inflammation and a positive anti SS-A antibody. She improved clinically with LDN therapy. The second case is a 66-year-old female with documented SS based on a history of dry eyes and dry mouth, joint pain, and elevated anti-SSA and anti-SSB antibodies whose joint symptoms responded to treatment with LDN.

Parotitis is a frequent disease in general pediatrics. Pneumoparotitis is a rare affection that belongs to differential diagnoses of parotitis, along with infections, lymphadenitis, autoimmune disorders, inflammatory conditions, vascular malformations or neoplasms. It is usually described in musicians using wind instruments or in other situations involving a Vasalva maneuver. We report the case of a 12 years old boy with severe idiopathic pneumoparotitis without any of these well-known causes and whose autoimmune familial background of Sjögren syndrome might be relevant.