In this case, the immune system attacks the organs that normally produce lubricating fluid, including the salivary glands in the mouth and the lacrimal glands in the eye. Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies, and lymphomas. When you have sjogren s syndrome, your eyes, mouth, and other parts of your body get dried out. Jun 14, 2018 although rare, localized amyloidosis can sometimes appear as a consequence of sjogrens syndrome, according to a case report from south korea the study, primary sjogren syndrome diagnosed simultaneously with localized amyloidosis of the lacrimal gland. The coexistence of sjogrens syndrome and hashimotos. This rare syndrome harbors mutations in the aldh3a2 gene located on chromosome 17p11. This is in accordance with available literature 4, 11, 12, 15. Chronic erythematous candidiasis on the dorsal surface of the tongue, and bilateral angle cheilitis.
It has been the subject of some more recent publication over the last decade,28 some suggesting a relationship to lupus erythematosus,9,10 others connective tissue diseases as well as. Primary sjogren syndrome pss rarely occurs in children. Results of this clinical report, with a 6year followup, showed that this patient diagnosed with sjogrens syndrome and rheumatoid arthritis could be treated successfully with dental implants and a fixed prosthesis. A case report about the coexistence of primary ss and euthyroid hashimotos thyroiditis ht in a. Middle aged females are commonly affected, female to male ratio being 9. Case report lrnf sjogrens syndrome complicated with iga. Sjogrens syndrome share this page sjogrens syndrome definition. It is an elaborate involvement of the lacrimal and salivary glands, which eventually lead to keratoconjunctivitis sicca and xerostomia.
Follicular lymphoma development in primary sjogrens syndrome. In addition, fatigue and musculoskeletal pain, often described as aching, is very common. Sjogren s syndrome is the second most prevalent autoimmune rheumatic disease. The case report presents the prosthetic management of a 47 year old female patient with sjogrens syndrome using a full mouth implant retained fixed hybrid prosthesis. Pdf sjogren syndrome is chronic, systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands. Although ss is epidemiologically encountered in middleaged and old females 9. Pdf article info abstract sjogrens syndrome commonly known as sicca syndrome is another representative of long lasting chronic. With this case report, we intend to emphasize the importance of an early diagnosis of this disorder, along with.
Common etiologies of acute acquired cerebellar ataxia include cerebrovascular diseases, toxin or. Sjogrens syndrome is a multisystem involving autoimmune disease. We present the case of a middle aged female with sjogrens syndrome ss and neuromyelitis optica spectrum disorders nmosd who had a rapidly declining neurological illness. On further evaluation she was diagnosed as a case of primary sjogren. A case report of rem sleep behavior disorder, behcets. This means that your immune system attacks parts of your own body by mistake. Those affected are at an increased risk 5% of lymphoma. Case report sjogrenssyndrome,vasculitis,with monoclonal 1gm. Sjogrens syndrome can present with glandular or extraglandular manifestations. We report the case of a 30yearold woman, with a past history of dry eyes and mouth, who presented a severe cerebellar ataxia worsening over 4 years associated with tremor of the limbs and the head. Sjogrens syndrome, like other autoimmune diseases, can be difficult to diagnose definitively, and often relies on a handful of signs and symptoms that can vary substantially from case to case. Progressive cerebellar degeneration revealing primary. Sjogrens syndrome involves inflammation of glands and other tissues of the body resulting in decreased production of tears and saliva.
Vmg medical college, maharashtra university of health sciences, solapur, maharastra, india author for correspondence abstract sjogrens syndrome is an autoimmune disease. Here we reported a case of giant coronary aneurysm in a 58yearold woman with a background of rheumatoid arthritis and sjogren syndrome history. According to our knowledge, this is the third case documenting the cooccurrence of gm and sjogren s syndrome. The accurate diagnosis of ss was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of antissa. In our study, we will report a case of thymic hyperplasia associated with pss cured by thymectomy.
Laboratory examination demonstrated hypokalemic rhabdomyolysis caused by distal renal tubular acidosis. This leads to dryness of the main mucosal surfaces such as the mouth, eyes, nose, pharynx, larynx, and vagina. Primary sjogrens syndrome has a high incidence of 9. Bowman sj, pillemer s, jonsson r, asmussen k, vitali c, et al. This is a report of a patient with acute onset cerebellar ataxia for one month. Such varied histology is likely to account for the var ied clinical presentation. We report a case of primary sjogrens syndrome ss with cutaneous leukocytoclastic vasculitis and iga nephropathy. Rhabdomyolysis presenting with severe hypokalemia as the first manifestation of sjogren s syndrome is rare. You may have dryness in other places that need moisture, such as your nose, throat, and skin. Sjogrens syndrome in a patient with ulcerative colitis. Adolescent sjogrens syndrome presenting as psychosis. Sjogrens syndrome is an autoimmune disorder characterized by dry eyes kerato conjunctivitis sicca, dry mouth xerostomia and glandular destruction parotid and lacrimal glands. Behcets disease and sjogrens syndrome is an autoimmune disorder from which many systems of the body can suffer. Macrophage activation syndrome, a rare complication of primary.
We report a case of a 59yearold woman who presented to our department with severe weakness of all limbs. The aim of this paper is to describe a rare case report of a pediatric patient with secondary sjogrens syndrome sss. Oral rehabilitation and management for secondary sjogrens. Abnormal lipid accumulation in the membranes of the skin and brain results in cutaneous and neurologic manifestations. Sjogren s syndrome, vasculitis, and cryoglobulinaemia 487 2 grossman j, abraham g n, leddy j p, condemi j l. Sjogren s syndrome causes increased levels of il1ra in csf suggesting increased activity in the interleukin 1. Sialendoscopy for recurrent parotitis associated with. Other immunosuppressive drugs may also be used particularly if sjogrens is associated with other diseases. Current diagnostic criteria for sjogrens syndrome developed by the american college of rheumatology include the presence of antinuclear antibodies, rheumatoid factor, antiro or antila autoantibodies. Sjogren s syndrome ss mayoccur in the absence primary ss or presence.
Peripheral neurological complications in primary sjogrens syndrome pss seem the most common, however the involvement of central nervous system cns. Sjogren syndrome is chronic, systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands. Initial and subsequent complete blood count and electrolyte values day 1 three after months later minerolocorticoid theraphy sodium mmoll 142 127 6 potassium mmoll 4. Cerebellar ataxia represents a rare and severe complication of sj. The present study reports an atypical case of ss in a 25yearold wife who was closely followed up for three years.
Xeiad cerrag this paper reports a malignant transformation of sjogrens syndrome to extraglandular follicular lymphoma in a 33yearold female. Case presentation a 55yearold woman complained chest distress, dryness of the oral cavity and eyes for 3 months. Sjogrens syndrome share this page sjogren s syndrome definition. Vaginal lubricants, such as replens, can be used for vaginal dryness. Treatment for sjogrens syndrome can involve medication such as hydroxychloroquine which is an antimalarial and used in sjogrens syndrome to modify and slow down the immune systems attack on the moisture producing glands. Sjogren s syndrome ss, a chronic autoimmune systemic disease affecting middle aged women, is characterized by lymphocytic infiltration of the salivary and lachrymal glands resulting in dry eyes. Researchers hope their study will increase awareness about the multitude of ways sjogrens syndrome may be recognized, and subsequently improving the diagnosis. A case report costa sm, perdigao ag, luz wc and piazera fz medical department, brazil introduction sjogrens syndrome ss is a dysfunction of the exocrine glands in response to lymphocytic infiltration and deposition of immune complexes, which leads to a chronic condition and a progressive glandular destruction. The clinical and theoretical implications are discussed.
Autoimmune lymphoproliferative syndrome alps is a rare disorder of the blood, estimated at around 500 cases worldwide. A rare clinical presentation of sjogrens syndrome, was. Jun 28, 2018 severe muscle weakness in all four limbs may be a symptom of sjogrens syndrome, a case report contends. A case report of prosthetic rehabilitation with 6year followup marcosdemendoncainvernici, 1 amandafingerstadler, 2 gastaovalenicolau, 3 mariaangelanavalmachado, 3 antonioadilsonsoaresdelima, 3 andmariliacompagnonimartins 3. Sjogrenssyndrome is an autoimmune disorder characterized by dry eyes kerato conjunctivitis sicca, dry mouth xerostomia and glandular destruction parotid and lacrimal glands. The use of osseointegrated dental implants and the design of the superstructure showed.
Here we reported a patient with a history of behcets disease and sjogrens syndrome in which rem sleep behavior disorder rbd was then detected by polysomnographic psg monitoring. Initial manifestations may be mild, and years may elapse from the initial manifestations to the full blown development and recognition of the syndrome. Treatment skin and vaginal dryness patients should use skin creams, such as eucerin, or skin lotions, such as lubriderm, to help with dry skin. Objectives to describe the sialendoscopic findings for a pediatric patient with recurrent bilateral parotitis associated with sjogren syndrome and to assess the therapeutic potential of sialendoscopy in this patient population. Ss is more prevalent in women than in men affects 0. Giant coronary aneurysm in a woman with sjogrens syndrome.
Nikitakis ng, rivera h, lariccia c, et al primary sjogren syndrome in childhood. A 26yearold mauritanian and berber woman was followed for primary sjogrens syndrome. Case report in the early 1900s, henrik sjogren and henri gougerot were the first to discovery the sjogrens syndrome. We present a case seen at our institute who was not diagnosed with sjogrens syndrome prior to her initial admission tobon et al. Sjogrens syndrome articles case reports symptoms treatment, ireland.
Neurological involvement has been reported in up to 80% of adults with primary sjogrens syndrome pss with psychiatric abnormalities including anxiety, depression, and cognitive dysfunction being common. Careful intraoral and extraoral examinations revealed. Psychosis due to pss has been reported in adult patients but has never been previously reported in the adolescentpediatric literature. Case report a 58yearold woman with a background of rheumatoid arthritis and sjogren syndrome history was referred to our hospital because chest pain once.
Review article kikuchifujimoto disease associated with sjogrens syndrome. In sjogren s syndrome, it attacks the glands that make tears and saliva. Sjogrens syndrome is a chronic autoimmune disorder that causes the inflammation of the lacrimal and salivary glands, resulting in dryness of the eyes and mouth. Sjogrens syndrome articles case reports symptoms treatment, germany. She was positive for antissa and antissb autoantibodies and presented with stinging of the eyes, the sensation of a foreign body in the eye, and reduced salivary secretion dryness of the mouth and altered sense of taste. The purpose of this report is to describe two patients with biopsyproven sjogrens syndrome lacking these autoantibodies but identified by antibodies to salivary gland protein 1. In sjogren syndrome, there are beehive or cystic alterations that are hypointense in t1weighted sequences and hyperintense on long tr. Case report international journal of clinical rheumatology 2018 volume, issue 4. Sjogrens syndrome articles case reports symptoms treatment, uk. It is characterized by a dysregulation of tcells in the immune system, and is caused by a defect in the process that. The only signs considered markers for sjogrens are antissa and antissb antibodies, and while antissb is more. Secondary sjogrens syndrome is associated with other autoimmune rheumatic diseases moutsopoulas and tzioufas, 2015. Sjogren s syndrome concurrently with ibd has very rarely been reported and seems to follow ibd diagnosis. Neurological examination showed reduced pinprick and absent vibration sensations in the lower limbs.
Antisalivary gland protein 1 antibodies in two patients. We present a case of a 67yearold woman admitted from the neurology clinic for further investigations of progressive ataxia and sensory symptoms. Sep 07, 2014 sjogren syndrome is chronic, systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands. Treatment directed toward alleviating the fatigue and pain associated with sjogrens is currently very limited. Report of a workshop help on 23 march 2000 at oxford, uk. A case report, was published in the journal medicine. We reported a case of sjogren syndrome with dry eyes symptom, polyathralgia, and fatigue. The patient was a 50yearold woman with newly diagnosed sjogren syndrome. Here, we would like to report a case of a patient with syringomyelialike syndrome in nmosd complicated with sjogrens syndrome. In 11month time, the intensity of lymphocytic infiltrates within salivary glands was much greater than was the case in 20. Oral surg oral med oral pathol oral radiol endod 2003, 96. Sjogrens syndrome with acute cerebellar ataxia and. Sjogrens syndrome uk pdf ppt case reports symptoms. Review article kikuchifujimoto disease associated with.
Results a yearold female presented with recurrent bilateral parotitis which was increasing in frequency. This clinical paper describes the management, treatment, and 6year followup of a patient diagnosed with ss type ii, who uses corticosteroids and antihyperglycemic drugs. Sjogrens showgrins syndrome is a disorder of your autoimmune system. Neuromyelitis optica nmo is a rare relapsing autoimmune disease of the central nervous system which is sometimes found in association with other autoimmune disorders including sjogrens syndrome. Sjogren 1 first reported a syndrome of xerostomia, keratoconjunctivitis sicca, and rheumatoid arthritis in 1933. Pdf sjogrens syndrome ss is a chronic, systemic autoimmune disease characterized by keratoconjunctivitis and xerostomia results from. Rare case of amyloidosis secondary to sjogrens syndrome is. Nevertheless, this case study demonstrated that pregnancy had totally recovered the glandular architecture of major salivary glands which was relapsed three months after delivery. Unexpected presentation of accessory breast cancer presenting as a subcutaneous mass at costal ridge. During embryogenesis, bilateral thickening of ectoderm from anterior axillary folds to inguinal folds, called mammary ridges or milk lines, develops into breast tissues. Sep 28, 2019 the aim of this report is to describe the clinic and physiologic alterations in this primary sjogrens syndrome case, and the therapeutic response to immunosuppressive treatment with rituximab, through a retrospective analysis of the clinical manifestations and pss biomarkers. Sjogrens syndrome in a patient with ulcerative colitis and. Sjogren s showgrins syndrome is a disorder of your autoimmune system. While not a cure for this autoimmune condition, modifying your diet can.
Sjogrens syndrome american college of rheumatology. Bcell lymphoma as a complication of sjogrens syndrome. Multiple limb weakness may be sign of sjogrens, case report says. A case report mohammed ayedh alqahtani prosthetic dental science, college of dentistry, king saud university, riyadh, saudi arabia abstract the case report presents the prosthetic management of a 47 year old female patient with sjogrens syndrome using a full mouth implant retained fixed hybrid prosthesis. Neuromyelitis optica spectrum disorder nmosd is a rare central nervous system cns autoimmune disease that can present as the initial manifestation in less than 5%. Herein we report a highly complex and interesting case of gm in a young female with sjogren s syndrome. Sjgrens syndrome, either primary or secondary, has been defined as an autoimmune epithelitis characterized by lymphocytic glandular infiltration and various extraglandular manifestations. As such ss should be considered as differential diagnosis in patients presenting with sensory ataxia. A rare case of sjogrens syndrome associated with intracranial. We describe a case of a 43yearold woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, raynauds phenomenon and dysautonomia. Complement c3 and c4, erythrocyte sedimentation rate esr, c.
Implant supported fixed hybrid prosthetic treatment in a. Jun 17, 2019 sjogrens syndrome is a chronic multisystem autoimmune disease characterized by inflammation and subsequent destruction of exocrine glands. By david ivanov, biochemistry and molecular biology, 2015 sjogrens syndrome, like other autoimmune diseases, can be difficult to diagnose definitively, and often relies on a handful of signs and symptoms that can vary substantially from case to case. She was previously diagnosed as sjogrens syndrome 3, and was. The coexistence of this syndrome with other autoimmune diseases, such as les, biliary cirrhosis, gastric achlorhydria, thyroiditis particularly hashimotos thyroiditis is reported in the literature. Primary sjogrens syndrome and the therapeutic response to. Other symptoms can include dry skin, vaginal dryness, a chronic cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. Sjogrens syndrome is an autoimmune condition that can occur at any age, but is most common in older women. A 25yearold female visited our outpatient clinic in april 20 with badly decayed teeth.
A case report of sjogren syndrome manifesting bilateral basa. A 29yearold female with history of sjogrens syndrome for the past 4 years presented to our hospital with complaints of. Sjogrens syndrome with acute cerebellar ataxia and massive lymphadenopathy. Syringomyelialike syndrome in neuromyelitis optica spectrum. We report a case of sls in an 11monthold girl of lebanese and mexicansyrian ancestry who presented with ichthyosis, developmental delay, and spasticity. A 45yearold hispanic woman presented with complain of double vision on horizontal and vertical gaze, which started 2 weeks prior to admission. Sjogren larsson syndrome introduction sjogren larsson syndrome sls is a rare autosomal recessive, neurocutaneous disorder. Hlab8 dr3,although dr4 is more closely associated with syndrome occuring with raynauds phenomenon antibodies to the ro antigen occur in excess in relatives of pts with sjogrens syndrome. A case report of sjogrens syndrome vijayalaxmi kanthe department of general medicine, dr. Case report management of sjogren s syndrome patient.
Sai shankar2 aarupadai veedu medical college and hospital, pondicherry, india abstract. There have been several studies showing a strong connection between systemic rheumatic disease and early atherosclerosis 22. Here we report a case demonstrating transition of vasculitis from nivd to mivd in serial biopsies, a rare and unusual occurrence. A case report of mesenteric panniculitis and primary. A 64old male han chinese, presented with three episodes of acute demyelinating processes in the central nervous system within 5 years. When the disease presents alone it is called primary sjogrens syndrome. It can also reduce muscle and joint pain and stiffness. Unusual presentation of sjogrens syndrome bmj case reports. There are only six cases in literature that describe development of dystonia with sjogrens syndrome ss. It is an elaborate involvement of the lacrimal and salivary glands. Case report sjogrenssyndrome,vasculitis,with monoclonal.
572 779 727 1547 277 79 456 687 1230 497 289 1482 640 1147 456 631 617 173 507 1137 347 825 763 330 699 959 591 1173 1351 268