Granulomatosis with polyangiitis (GPA) in a 76-year old woman presenting with pulmonary nodule and accelerating acute kidney injury
Published on: 20th January, 2020
OCLC Number/Unique Identifier: 8516756417
Granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis (AAV), is a rare disease with an often-occult presentation. It is more common in 4th and 5th decades of life but can be seen in all ages.
This case report details a 76-year-old female presenting with abdominal pain, generalized weakness, and malaise, who was found to have pulmonary nodules on chest imaging. Biopsy of the lung nodule showed organizing pneumonia. Initially, antibiotics were used to treat the patient. However, she developed acute renal failure a few days after presentation and found to have positive serum C-ANCA as well as elevated ANCA-PR3 serologies. A subsequent kidney biopsy demonstrated pauci-immune necrotizing and crescentic glomerulonephritis that was consistent with GPA and the patient was started immediately on combination immunosuppressive therapy, plasmapheresis, and hemodialysis.
GPA’s clinical and radiological presentation can mimic other common conditions such as pneumonia, malignancy, bacterial sinusitis, pulmonary tuberculosis, sarcoidosis, and urinary tract infection. Because of this, a high level of suspicion is required for early diagnosis and treatment to alter the high mortality rate in this disease entity. All forms of ANCA-associated vasculitis (AAV) should be in the differential diagnosis for all patients presenting with multiorgan system involvement particularly in individuals with pulmonary and renal manifesations.
A case of resistant hypercalcemia in a peritoneal dialysis patient with underlying Vitamin A toxicosis
Published on: 7th October, 2021
OCLC Number/Unique Identifier: 9305374138
Hypercalcemia in End Stage Renal Disease on Dialysis, is a frustrating complication for both medical staff and patients, and it may lead to vascular calcification, Calciphylaxis, and even aggravating cardiovascular disease, even in the absence of risk factors which can lead to early death [1], and correcting Hypercalcemia even in the absence of hyperphosphatemia is out most important to improve co-morbid conditions and reduce mortality, most common causes in end stage renal disease, includes high calcium dialysis bath, high dietary intake of Calcium rich food, exogenous intake of calcium products, or excessive intake of Vitamin D, underlying Sarcoidosis, rare causes need to be explored in resistant cases, including Vitamin A toxicosis, as being presented in this case.
Incidence and features of heparin-induced thrombocytopenia in mechanical heart valves and immunologic cardiac diseases
Published on: 5th January, 2022
OCLC Number/Unique Identifier: 9391755940
Background: Heparin-induced thrombocytopenia/thrombosis (HIT/T) is characterized by a fall in platelet count 5-10days after starting heparin therapy and is diagnosed with specific 4-T clinical features and laboratory tests. This complication is relatively common in Cardiothoracic surgery patients. Objective: To evaluate the positive and negative predictive value of various HIT laboratory tests and assess any correlation between HIT, the underlying diagnosis, underlying procedure, and mechanical cardiac devices. Patients and methods: The patient’s medical records were correlated with two laboratories HIT diagnostic tests, the pan-specific screening test with IgG, IgA, and IgM antibodies, followed by HIT specific IgG ELISA. Results: Total n = 80 patients were assessed, 48% (n = 38) were HIT screen pan-specific negative and 50% (n = 40) were HIT pan-specific positive and 2 cases were inconclusive. 17% (n = 14) were both pan-specific and specific HIT IgG ELISA positive. There were 5 atypical cases. One patient had Eosinophilic myocarditis and was HIT ELISA IgG neg. Argatroban was given on clinical grounds with successful recovery. One patient with Sarcoidosis had an aggressive course and received IV Immunoglobulin (IVIG) but succumbed secondary to liver failure. One patient progressed to gut ischemia and had surgical intervention but succumbed. Two patients with mechanical heart valves were on Argatroban but relapsed and responded to IVIG therapy. Conclusion: Our study indicates that 9/16 (> 50%) HIT-positive patients had valve replacement or cardiac devices suggesting that like knee arthroplasty there is a high incidence of HIT in patients with mechanical heart valves and cardiac devices and this warrants further prospective study.
Overlap of two unusual condition in childhood: hibernoma and central diabetes insipidus
Published on: 7th March, 2022
Central Diabetes Insipidus (CDI) results from the inability to secrete ADH secreted by the neurohypophysis system to control water-electrolyte metabolism. In the etiology of CDI in childhood, many congenital and acquired central nervous system (CNS) tumors (germinoma, pinealoma, craniopharyngioma, optic glioma, acute myeloid leukemia), infiltrative diseases (Langerhans cell histiocytosis, sarcoidosis), infections (meningitis, tuberculosis, encephalitis), autoimmune events, head trauma, idiopathic) can be responsible [1]. Hibernomas, which are very rare in childhood, may also rarely involve the central nervous system.
Follow-up of patients with sarcoidosis in an internal medicine unit from a hospital in Asturias, Spain. Analysis of extrapulmonary manifestations
Published on: 2nd September, 2022
OCLC Number/Unique Identifier: 9617326596
Sarcoidosis is a multisystem disease of unknown origin that is characterized by non-caseating epithelioid granuloma formation within various organs, mainly the lungs [1]. The objective of this study is the analysis of sarcoidosis patients affected by pulmonary manifestation alone or extrapulmonary expression.
A challenging case of cutaneous sarcoidosis with unusual findings in a Syrian woman: a case report and review of literature
Published on: 14th March, 2023
Sarcoidosis is a granulomatous disease that involves multiple systems. Cutaneous involvement can manifest in patients with sarcoidosis and can present with or without systemic involvement. We present a case of cutaneous sarcoidosis in a Syrian woman that showed improvement after a combination of methotrexate and prednisolone therapy. The patient had unusual chest radiography findings and developed an unexplained fissured tongue after 5 days of receiving methotrexate therapy. In addition, the patient developed indurated erythematous plaque and papules on her upper right arm at the same location as a performed biopsy, which increased the diagnosis of a newly formed scar sarcoidosis although she was receiving her treatment.
Delayed Diagnosis of Early-onset Sarcoidosis: A Case Report and Literature Review
Published on: 18th January, 2024
Background: Early-onset sarcoidosis is a rare systemic inflammatory granulomatous disease, distinguished by onset before the age of 4 and notably lacking pulmonary involvement. Unfortunately, the condition often shows clinical features similar to juvenile idiopathic arthritis, resulting in the misdiagnosis of numerous patients. This case report delves into the challenges associated with the delayed diagnosis of early-onset sarcoidosis, with a particular focus on the diagnostic methods employed to address this delayed recognition.Case presentation: A 15-year-old girl presented with a history of recurrent fever since infancy, accompanied by rash, arthritis, and joint deformity. Previously misdiagnosed with juvenile idiopathic arthritis, she underwent management with steroids and methotrexate, yielding no improvement. The diagnosis of early-onset sarcoidosis was made during adolescence based on serial examinations, comprehensive laboratory and radiological evaluations, and subsequent histopathology findings. Presently, the patient is receiving treatment with low-dose steroids and biologic therapy (Tocilizumab) and experiencing no disease progression.Conclusion: This case report underscores the importance of considering early-onset sarcoidosis in the differential diagnosis of pediatric patients exhibiting persistent arthritis from an early age. Early detection and treatment are crucial in averting complications and enhancing the overall quality of life.
Adalimumab in the Treatment of Complex Sarcoidosis-related Inflammatory Eye Disease: A Case Series
Published on: 21st May, 2024
Background: Sarcoidosis is a systemic granulomatous inflammatory disease that is associated with inflammatory eye manifestations such as uveitis, cystoid macular edema, and retinal vasculitis. Although Corticosteroids (CS) have traditionally been the mainstay of treatment, there is a clinical need and growing interest in exploring alternative therapeutic options for patients who are refractory to or intolerant of CS or require long-term steroid-sparing agents. Purpose: This case series aims to describe the effectiveness of adalimumab, an anti-tumor necrosis factor (TNF)-α monoclonal antibody, in the management of complex sarcoidosis-related inflammatory eye disease via reduction in CS dosage and ocular exam findings before and after initiation of adalimumab therapy.Method: A retrospective chart review of patients between 2010 and 2023 seen at our academic center’s rheumatology and eye clinics was conducted, with 5 patients meeting the inclusion criteria. Results: Most patients were able to lower, discontinue, or remain off oral CS, while all 5 patients demonstrated a reduction in uveitis activity, Cystoid Macular Edema (CME), and/or retinal vasculitis. Conclusion: These findings suggest a potential role for adalimumab as an effective and safe therapeutic option in the management of complex sarcoidosis-related inflammatory eye disease.
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