A diagnostic complexity in a female primigravida with corneal ulcer
A 20-yr-old female primigravida presented to the outpatient department with severe arthritis involving small joints of hands and feet, and a vision-threatening (6/60) marginal corneal ulcer in the left eye during postpartum (Fig 1). Although, the patient had a history of recurrent epistaxis in her 5th month of gestation and the occurrence of saddle nose (Fig. 1) in the 8th month, she ignored these symptoms. She had a normal full-term delivery and she did not receive any treatment during pregnancy. No history of hemoptysis, reduced urine output, fever, weight loss, weakness on one side of the body, or ear pain was reported.
General assessment revealed that the vital signs and the functioning of cardiovascular and neurological systems were normal. The results of laboratory investigations are as follows: Hb-12.2gm/dl, Tc-14800/dl with normal differentials, ESR-120mm/1st hr, urine protein- 75mg/day, negative RF and ANA antibodies, and positive c-ANCA. Chest X-ray examination and urine routine did not report any abnormal findings.
1 / 4
Which of the following is not a probable diagnosis for this patient's condition?
Based on the symptoms described, option D (Sjögren's syndrome) is the least probable diagnosis for this patient's condition.
2 / 4
Which laboratory finding is typical for Wegener's granulomatosis?
Wegener's granulomatosis, now referred to as granulomatosis with polyangiitis (GPA), is associated with the presence of anti-cytoplasmic antibodies (ANCA), specifically c-ANCA (cytoplasmic antineutrophil cytoplasmic antibodies). These antibodies are directed against proteins in the cytoplasm of neutrophils. The other options are not typically associated with Wegener's granulomatosis
3 / 4
Which of the following is a characteristic feature of Wegener's granulomatosis?
Wegener's granulomatosis often involves the respiratory tract, leading to pulmonary infiltrates and nodules.
4 / 4
Which of the following is not indicated for treating Wegener's granulomatosis?
The correct answer is ‘Infliximab,’ and it is a tumor necrosis factor (TNF) inhibitor. While it is used in the treatment of various autoimmune diseases, it is generally not considered a first-line treatment for Wegener's granulomatosis.
She was diagnosed to have vision-threatening marginal corneal ulcer due to WG. The patient was treated with intravenous pulse methylprednisolone (1 gm for 3 days) and cyclophosphamide (500 mg every 15 days for 6 cycles) followed by a tapering dose of methylprednisolone. Improvement in corneal ulcer was seen following medication. The patient is presently on methotrexate 15 mg/week (as steroid-sparing medication) and 5 mg of methylprednisolone and now the symptoms are minimal.
The case study has been adopted from our journal Internet Journal of Rheumatology and Clinical Immunology. For further details regarding the case discussion, please visit: https://www.chanrejournals.com/jr/index.php/rheumatology/article/view/43/version/53/html
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