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Granulomatosis with polyangiitis (GPA) is a small- to medium-sized blood vessel vasculitis that commonly affects the upper and lower respiratory tract, kidneys, eyes, nose, and ears. Pituitary involvement in GPA is rare, seen in about 1% of cases. Here, we discuss a young woman presenting with headache and diplopia in the setting of pituitary masses. Despite transsphenoidal resections and intravenous antibiotics, her symptoms progressed. She developed visual field defects, nasal crusting, and dyspnea with cavitary lung lesions and nodules. The diagnosis of GPA was established through pathology, radiographic findings, laboratory results, clinical history, and response to steroids and rituximab induction therapy.

Elevated Activated Partial Thromboplastin Time in the Setting of Prophylactic Unfractionated Subcutaneous Heparin Administration | Annals of Internal Medicine: Clinical Cases

Subcutaneous unfractionated heparin, or low–molecular-weight heparin, is commonly used for venous thromboembolism prophylaxis in hospitalized patients at increased risk for developing thrombosis. Generally, the prophylactic doses of these medications have poor bioavailability and do not reach therapeutic serum concentration levels to cause coagulopathy. Here, we report a case of a patient who had an elevated activated partial thromboplastin time while receiving prophylactic dosing of subcutaneous unfractionated heparin for venous thromboembolism prophylaxis.

Early-Onset Bevacizumab-Associated Knee Osteonecrosis in a Young Adult With Astrocytoma | Annals of Internal Medicine: Clinical Cases

We describe a rare case of knee osteonecrosis in a 22-year-old man with astrocytoma and neurofibromatosis type 1 after short-term treatment with bevacizumab and corticosteroids. Magnetic resonance imaging (MRI) identified infarctions and osteonecrosis of the distal femur and proximal tibia. Osteonecrosis associated with bevacizumab is typically limited to the jaw; this case highlights the potential for earlier, extra-jaw involvement. Clinicians should maintain a high index of suspicion for osteonecrosis in patients on vascular endothelial growth factor inhibitors, especially with concurrent steroid use, and consider prompt MRI in the evaluation of unexplained joint pain.

Idiopathic Granulomatous Mastitis: A Rare Disorder to Consider in the Differential Diagnosis of a Breast Mass | Annals of Internal Medicine: Clinical Cases

Idiopathic granulomatous mastitis (IGM) is a rare inflammatory breast condition that resembles infectious mastitis and malignancy, making diagnosis and treatment challenging. We report the case of a 32-year-old woman with a painful, enlarging left breast mass. Ultrasound-guided biopsy demonstrated cystic neutrophilic granulomatous mastitis. Her infectious work-up yielded negative results. The patient was treated with antibiotics that were then discontinued after biopsy and negative culture and she was discharged on steroid taper. Over the subsequent 18 months, she had steroid-dependent flares and, despite methotrexate and adalimumab, required a lumpectomy. This case highlights the need for early recognition of IGM in breast masses and the challenges in diagnosis, management, and care.

The Dark Side of Licorice Consumption | Annals of Internal Medicine: Clinical Cases

Licorice-induced apparent mineralocorticoid excess is a rare but reversible cause of hypertension and hypokalemia. Glycyrrhizic acid (GA), found in licorice, inhibits 11β-HSD2, increasing renal cortisol and activating mineralocorticoid receptors. We report a 50-year-old woman with episodic peripheral edema, hypertension, and hypokalemia. She was diagnosed with licorice-induced apparent mineralocorticoid excess caused by consumption of licorice root tea. Her intake exceeded the 75-mg GA threshold known to elevate blood pressure. Diagnosis is challenging as laboratory tests such as renin and aldosterone levels may normalize. This case highlights the importance of a thorough dietary history and clearer labeling of GA content in licorice-containing products.

Co-occurrence of Klinefelter Syndrome and Antisynthetase Polymyositis Complicated by Interstitial Lung Disease | Annals of Internal Medicine: Clinical Cases

A 15-year-old boy with a medical history of obesity and mild intermittent asthma presented with progressive respiratory distress despite initial treatment of suspected atypical pneumonia. The differential diagnosis was broadened to include an autoimmune cause, and work-up revealed an elevated creatine kinase level and a positive anti–Jo-1 titer, leading to a diagnosis of antisynthetase polymyositis. The patient improved with high-dose steroids and was transitioned to a steroid-sparing regimen before discharge. Fifteen months later, the patient was found to have Klinefelter syndrome. This case highlights the sex bias of autoimmune disease, which is important to consider even in phenotypically male patients.

A Case of Pancreatitis, Panniculitis, and Polyarthritis Syndrome in Pancreatic Acinar Cell Carcinoma | Annals of Internal Medicine: Clinical Cases

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare triad driven by an underlying pancreatic pathology that releases high levels of pancreatic enzymes into systemic circulation. We describe a patient who presented with erythematous skin nodules, painful swelling of multiple joints, and vague abdominal symptoms who was ultimately diagnosed with metastatic pancreatic acinar cell carcinoma. Early diagnosis of PPP syndrome remains challenging because rheumatologic findings often overshadow gastrointestinal symptoms. Timely diagnosis is crucial, because treatment of the underlying pancreatic disease improves symptoms and minimizes long-term complications.

Resolution of Hypokalemia and Hypotension With Tacrolimus and Extrarenal Transplantation in Gitelman Syndrome | Annals of Internal Medicine: Clinical Cases

Gitelman syndrome (GS) is characterized by genetic mutations in the sodium chloride cotransporter (NCC), leading to renal potassium, sodium, and magnesium wasting. Calcineurin inhibitors recapitulate familial hyperkalemic hypertension, with gain of function of the NCC. Whether calcineurin inhibition can overcome fluid/electrolyte dyscrasias in GS is unknown. We describe a patient with GS initiated on tacrolimus, a calcineurin phosphatase inhibitor, after orthotopic heart transplantation. Potassium supplementation and potassium-sparing diuretics were discontinued with no consequential hypokalemia, and hypotension and hypomagnesemia also improved. This case provides proof of concept that calcineurin inhibitors may help overcome clinical sequelae of loss of function of NCC in GS.

Nasal Flaring in an Unconscious Child With Severe Malaria: Compensation Reflex for Metabolic Acidosis | Annals of Internal Medicine: Clinical Cases

Kussmaul breathing is a clinical sign of metabolic acidosis. Inspiratory flaring of the alae nasi may accompany these respirations, enhancing compensatory CO2 exhalation. We describe a child with severe falciparum malaria, coma, and metabolic acidosis. We discuss a ventilatory reflex, localized in the brainstem, which senses rising H+/CO2, and leads to a compensatory coordinated unconscious response, which includes nasal flaring.

Exploring Azathioprine as a Trigger for Lymphomatoid Granulomatosis | Annals of Internal Medicine: Clinical Cases

Lymphomatoid granulomatosis is a rare Epstein–Barr virus–associated B-cell lymphoproliferative disorder. The disease commonly manifests with respiratory symptoms, often leading to the discovery of bilateral pulmonary nodules on imaging. We present a 77-year-old woman with a history of Sjögren syndrome who was receiving azathioprine, was found to have bilateral pulmonary nodules initially concerning for metastatic disease, and who was later diagnosed with lymphomatoid granulomatosis. Azathioprine was discontinued, and the patient was observed without receiving chemotherapy. Subsequent scans revealed a decrease in disease burden. Awareness of this rare but serious side effect is important for all clinicians managing patients who are receiving long-term immunosuppressive therapy.

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