Macrophage Activation Syndrome Presents as Initial Manifestation of Lupus in an Adult Female.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the immune system erroneously attacking healthy tissues and organs. SLE has a wide variety of clinical presentations. The signs and symptoms of SLE are very well-known, though rare presentations could occur that require early clinical attention. Macrophage activation syndrome (MAS) is a severe and life-threatening condition in which the immune system becomes overactive, leading to the excessive stimulation and proliferation of immune cells. MAS can occur as a primary immune disorder, which is not very common. It can also happen secondary to a wide variety of pathological conditions, which include infections, malignancies, autoimmune, and rheumatologic disorders. In rare cases, SLE can present with overlapping features of MAS, further complicating the clinical picture, and may require specialized management. Early recognition and intervention of this overlap are essential for improving outcomes, as delayed diagnosis and treatment can lead to significant morbidity and mortality. Here, we present a case of a young adult female who was diagnosed with SLE with the initial presentation of MAS in the form of fever, splenomegaly, cytopenia, and hemophagocytosis.

Chorea as an initial and solitary manifestation of systemic lupus erythematosus with antiphospholipid syndrome in an elderly man.

Chorea can be an initial manifestation of systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). It has been mostly described in younger female adults in association with other manifestations of SLE. When chorea appears as an initial and only manifestation in SLE/APS patients, the establishment of the correct diagnosis is difficult, and it may be initially attributed to a more common aetiology. Here we report an elderly man who presented with a new onset of right-sided chorea without other clinical manifestations of SLE/APS. He started on steroids a year later, however, there was no improvement. His chorea was symptomatically managed along with aspirin, and hydroxychloroquine as he refused to be on additional immunosuppression. Anticoagulation was relatively contraindicated, and also not favoured by this patient; therefore, aspirin was initiated. Even in elderly patients, once the common etiologies of chorea have been worked up, we suggest doing a rheumatological evaluation. Early diagnosis and prompt treatment can prevent persistent neurological abnormality.

The Mystery of Clopidogrel-Associated Inflammatory Arthritis.

Clopidogrel is an antiplatelet medication that plays an important role in the management and prevention of thrombotic vascular events in patients with acute coronary syndrome (ACS) and ischemic stroke. We report a case of a male patient who received a maintenance dose of clopidogrel as part of stroke treatment and developed inflammatory arthritis after five days of starting the medication. He underwent extensive evaluation and testing to explore other common causes of inflammatory arthritis, including autoimmune etiologies. None of the test results were helpful, and we hypothesized that his arthritis was induced by clopidogrel. Discontinuing this agent resulted in the complete resolution of the patient's symptoms. Since medication-induced arthritis is a diagnosis of exclusion, these patients should undergo a complete workup for inflammatory arthritis. If possible, a risk-benefit analysis of dual antiplatelet therapy (DAPT) in ischemic stroke patients with a prior history of rheumatoid arthritis (RA) should be done in collaboration with neurology.

Certolizumab-induced liver injury: as assessed for causality by the updated RUCAM.

We report a case of a woman in her 30s with a history of cholecystectomy, hypertension, type 2 diabetes and rheumatoid arthritis on methotrexate and certolizumab who presented with epigastric pain radiating to the right upper quadrant. Laboratory findings revealed significantly elevated liver enzymes consistent with hepatocellular liver injury. The hepatocellular pattern of liver injury, negative autoimmune serologies and improvement on cessation of certolizumab were consistent with drug-induced hepatotoxicity. We used Roussel Uclaf Causality Assessment Method to assess the likelihood of drug-induced liver injury that showed a score of 7, this was in line with a probable causality grading. Although the patient had a history of methotrexate use, the onset of symptoms and transaminitis coincided with the recent initiation of certolizumab, which was the trigger of hepatocellular injury. Liver enzymes also remained normal after the reinitiation of methotrexate on follow-up.

The rise and fall of troponin in a patient with polymyositis.

Troponins are an excellent sensitive marker for myocardial ischaemic damage. However, there are several non-ischaemic cardiac and non-cardiac reasons for troponin elevation. Many cases of troponin T elevation and some troponin I cases have been reported in the literature due to inflammatory muscle disease. Here, we report a woman in her 50s who initially presents with fatigue and weakness, and is found to have elevated troponin T. The patient was appropriately worked up for cardiac causes with ECG and echocardiogram. She had positive antinuclear antibodies, antineutrophil cytoplasmic antibody and myositis panel. The elevation of troponins was attributed to polymyositis and treated with methotrexate and prednisone with recovery of patient's symptoms. This article emphasises the struggle of diagnosis in a patient with no reported medical history, having low to moderate risk of silent myocardial infarction.

Hemophagocytic Lymphohistiocytosis in a Patient with Hodgkin Lymphoma, HIV, and Epstein-Barr Virus.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. It occurs in many underlying conditions and all age groups due to severe and uncontrolled inflammatory reactions, with the resultant overproduction of immune cells and cytokines. This leads to multi-organ damage (if not detected early and treated properly) with a mortality of more than 55%. We present a case of a 38-year-old male patient who presented with HLH with concurrent HIV/AIDS, and Epstein-Barr virus (EBV)-related Hodgkin lymphoma. We aim to emphasize the importance of considering HLH and cancer in patients with HIV/AIDS.

Rare But Fatal: Hemophagocytic Lymphohistiocytosis (HLH) With Acute Acalculous Cholecystitis.

Acalculous cholecystitis is an acute inflammatory disease of the gall bladder with high morbidity and mortality rate. It can be seen in trauma, burns, sepsis, total parenteral nutrition, prolonged fasting, and autoimmune diseases. However, there are very few reports of acalculous cholecystitis with macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) in patients with underlying rheumatic/autoimmune disorders. Here we report a 23-year-old male with a past medical history of granulomatosis with polyangiitis who presented with fever, weight loss, and pancytopenia. A comprehensive infectious evaluation was done including bacterial cultures and viral and fungal serologies. Repeat abdominal imaging obtained later due to developing abdominal pain raised concerns for acute acalculous cholecystitis. Despite aggressive management of sepsis, the patient continued to decline clinically. HLH was suspected when the patient was found to meet the clinical criteria with fever, splenomegaly, cytopenia, hypertriglyceridemia, elevated liver function tests, hypofibrinogenemia, and ferritin of 22K ng/mL, absent NK cell activity, and elevated soluble CD25 receptor levels. Bone marrow biopsy did not reveal hemophagocytosis. Intravenous methylprednisolone was started and the patient showed remarkable clinical improvement with a decrease in all inflammatory markers and did not require any surgical intervention. On the review of the literature, we were able to identify four female patients with underlying adult-onset Still's disease and Kikuchi disease who presented with HLH along with acalculous cholecystitis likely triggered by flare. Our male patient presented with HLH and acute acalculous cholecystitis. He had a history of granulomatosis polyangiitis (GPA) that remained in remission. Hypersecretion of pro-inflammatory cytokines and cytotoxic cells in HLH promotes ischemia of the gall bladder wall. Early initiation of immunosuppressive therapy under careful observation can prevent surgical intervention and mortality in these patients.

Dot-in-Circle Sign - A Diagnostic MRI Sign for "Madura Foot".

Mycetoma is a slowly progressive localised, granulomatous, subcutaneous tissue infection common among adult males aged 20 - 50 years. The causing agents include both bacteria (actinomycetoma) and fungi (eumycetoma). This chronic infection was described in literature as Madura foot and eventually mycetoma, due to its etiology. Early clinical diagnosis before the appearance of sinuses and grains (aggregates of organism surrounded by granulation tissue, which are discharged from the draining sinuses) is difficult. Early diagnosis is important due to the therapeutic implications. Although biopsy and microbiological cultures provide definitive diagnosis. Delay in diagnosis may lead to amputation of the affected part. The recently described "dot-in-circle" sign on magnetic resonance imaging (MRI) is easy to recognise and highly specific. We present a case of histologically proven mycetoma with characteristic MRI features.

Multidetector CT Patterns of Peritoneal Involvement in Patients with Abdominopelvic Malignancies.

OBJECTIVE: To determine the patterns of peritoneal involvement in patients with abdominopelvic malignancies. STUDY DESIGN: Retrospective observational study. PLACE AND DURATION OF STUDY: Shifa International Hospital, Islamabad, Pakistan, from May 2004 to May 2012. METHODOLOGY: Two hundred and three patients with histopathologically proven abdominopelvic malignancies with peritoneal involvement who underwent contrast-enhanced CT abdomen and pelvis were identified through electronic data base system and were included in this study after ethical committee approval. Peritoneal disease pattern, predominant sites of involvement and associated findings of ascites, lymph nodes and metastasis were assessed. Patients with tuberculosis and lymphoproliferative disorders were excluded. RESULTS: The malignancies showing peritoneal involvement, in decreasing order of frequency, were ovarian cancer (n=118), colorectal cancer (n=45), pancreatic cancer (n=11), gastric cancer (n=7), endometrial cancer (n=6), gallbladder/ cholangio-carcinoma and hepatocellular cancer (n=5 each), cervical cancer (n=3), renal cell carcinoma (n=2) and transitional cell urinary bladder cancer (n=1). The most common pattern of peritoneal involvement was mixed in 79 patients (39%), omental caking in 74 patients (37%) and nodular deposits in 50 patients (24%). The most common sites of peritoneal involvement were pelvic peritoneum followed by greater omentum. CONCLUSION: Peritoneal involvement is the commonest with ovarian and colorectal carcinoma. Mixed pattern of peritoneal disease was most frequently seen in these patients followed by omental caking and nodular peritoneal deposits.