Primary Sjogren's syndrome presenting as an isolated severe autonomic dysfunction treated with steroids.

Primary Sjogren's syndrome (pSS) is an autoimmune connective tissue disorder with multisystem manifestations. We here report a previously healthy woman who presented with autonomic dysfunction in the form of severe dizziness without any apparent sensory neuropathy. Detailed history and examination revealed the signs and symptoms of Sjogren's syndrome such as constipation and dry eyes and mouth, following which anti-SSA and SSB antibodies were found to be positive. Finally, a diagnosis of pSS was established after ruling out all the other causes of autonomic dysfunction in addition to the clinical and laboratory evidence. The patient was treated with the maximum doses of midodrine and fludrocortisone, yet no progress was noticed. Hence, a trial of steroids was started and she showed a significant clinical improvement. Our patient presented with pure autonomic failure associated with Sjogren's syndrome, making it an extremely rare entity.

Spontaneous muscle haematoma in a patient with cirrhosis.

Decompensated liver disease is associated with alterated haemostasis that can either lead to spontaneous bleeding or development of thrombosis. Alcohol consumption coupled with advanced liver disease favours spontaneous bleeding. There have been only few documented cases of spontaneous muscle haematoma (SMH) in patients with cirrhosis. The pathogenesis of SMH is hypothesised to be multifactorial and it has been seen in patients on anticoagulation or with haemostatic disorders. We report a case of alcohol-related cirrhosis presenting with an expanding, voluminous haematoma in the intermuscular plane between the trapezius and the teres major muscles. This patient also had a retroperitoneal haemorrhage, clinically evidenced by the Grey Turner's and Cullen's signs. Haemorrhage was confirmed radiologically by CT. The patient was managed in an intensive care facility and treated with multiple blood products, including packed red blood cells, fresh frozen plasma and cryoprecipitates. However, as his clinical condition deteriorated, he required surgical intervention by incision and drainage, followed by evacuation. Early identification of coagulopathy and aggressive treatment are essential in these cases of cirrhosis to avoid unfavourable outcomes.

Unusual and rare case of generalised lymphadenopathy: Kimura's disease.

We report a case of Kimura's disease in a 65-year-old woman who presented with generalised itching, abdominal pain, facial puffiness, difficulty in swallowing and loss of appetite. She was found to have generalised lymphadenopathy and a fine-needle aspiration cytology initially done revealed 'reactive lymphadenitis' which was inconclusive. PET-CT done showed features suggestive of lymphoma. Hence, lymph node biopsy was done for confirmation and incidentally after immunohistochemistry staining it turned out to be Kimura's disease. High-dose steroid therapy was started and patient showed dramatic clinical and symptomatic improvement. Kimura's disease almost always presents as cervical lymphadenopathy and usually never causes compressive symptoms. Our patient presented with compressive symptoms and generalised lymphadenopathy which is a rarity.