Faculty Development Case of the Month: June 2024

Monthly Case from the Department of Medicine 
An Unusual Case of Generalized Lymphadenopathy, Right Upper Quadrant Pain, & Transaminitis 

 

Submitted by:
Shae Chambers
Medical Student
Department of Medicine 
Emory University School of Medicine 

Ashley Urrutia, MD, MPH
Assistant Professor of Medicine
Division of General Internal Medicine 
Emory University School of Medicine

 

STORY AND CASE
A 43-year-old Hispanic woman with a past medical history of hypertension and cholecystectomy presented to the emergency department with right upper quadrant (RUQ) pain and pruritic rash localized to the posterior surfaces of the upper arms. 

 She described the RUQ pain as dull and non-radiating, first occurring after eating a fatty meal 3 days prior. She endorsed a one-month history of bilateral, pruritic rash on the upper extremities as well as bilateral wrist pain for the past 3 months and bilateral knee pain for the past year. She moved one year ago from Peru. She is married and lives with her husband and niece. She denied sick contacts or new sexual partners. Family history was positive for arthritis and “thyroid problems” on the maternal side. She noted a recent history of receiving a contraceptive injection at a beauty shop 1 month ago, but otherwise did not take any medications or supplements regularly. Physical exam was notable for severe RUQ tenderness to palpation, cervical lymphadenopathy, as well as dusky, flat, non-blanchable lesions on the posterior surfaces of upper arms bilaterally ranging from 8mm to 12mm with some excoriations but no skin breakage or ulceration.  

Work-up on admission showed transaminitis with elevated AST and ALT values of 115 and 136 respectively, as well as an elevated alkaline phosphatase of 421 and elevated gamma-glutamyl transpeptidase of 264. Her HIV, RPR, and hepatitis serologies were all negative. Her initial rheumatologic work-up showed an ANA titer of 1:1280. Urine studies revealed proteinuria and hematuria. A right upper quadrant ultrasound showed mild dilation of the proximal common bile duct with no visualized biliary lesions. A CT of the abdomen/pelvis without contrast showed multifocal cervical, supraclavicular, axillary, mediastinal, and retroperitoneal lymphadenopathy. An x-ray of the bilateral wrists showed subchondral cystic changes involving the ulnar aspect of the lunate bilaterally.  

What’s the Diagnosis?
Systemic lupus erythematosus (SLE) 

ANSWER AND EXPLANATION
Further rheumatologic testing showed positive SSA (Ro) antibody, anti-histone antibody, RNP antibody, smith antibody, and ds-DNA antibody which were most concerning for a diagnosis of systemic lupus erythematosus (SLE).

Two days following the initiation of 20mg prednisone treatment, a 24-hour urine collection demonstrated an elevated protein excretion of 1,575mg/24hr (ref 10-100mg/hr). A kidney biopsy was performed which showed patchy chronic interstitial inflammation, tubular atrophy, and interstitial fibrosis most concerning for class V lupus nephritis. 

DISCUSSION
Findings of transaminitis are not uncommon among newly diagnosed SLE patients. [1] The causes of these liver function abnormalities may range from drug toxicity to comorbidities like fatty liver and chronic hepatitis B or C viruses. Liver involvement may also be a result of lupus itself called lupus hepatitis, the prevalence of which has been reported to be between 3-23%. [2] 

In the case of our patient, we hypothesize that findings of transaminitis, bile duct dilation, and RUQ pain may have been the result of compressive lymphadenopathy as seen on CT to be most predominant in the cervical, supraclavicular, axillary, mediastinal, and retroperitoneal regions.  

Her upper extremity rash appeared as dusky, flat, non-blanchable lesions. Typical rashes associated with lupus, which are included in the disease diagnostic criteria, include malar rash across the nose and cheeks, red or purple scaly rash, and photosensitive rash. Dermatology performed a punch biopsy which showed nonspecific inflammatory infiltrate most concerning for potential granulomatous dermatitis associated with connective tissue disease. The subchondral cystic changes noted on wrist x-ray maybe be related to SLE, as cystic bone changes can be seen in a subset of SLE patients. [3]  

The differential for generalized diffuse lymphadenopathy is complex and initial suspected etiologies may include lymphoma, syphilis, and tuberculosis, all of which were negative in the initial work-up of our patient. Although lymphadenopathy is not included in the current accepted clinical criteria for SLE diagnosis, past studies have shown that it is a common finding among SLE patients. In a 1954 case series by Harvey et al published, 105 of 138 patients with SLE had generalized lymphadenopathy, with cervical and axillary nodes being the most common. [4] Another study in 1964 reviewed 520 cases of lupus over 13 years found lymphadenopathy in approximately 56% of patients. [5] 

CITATIONS  

1. Imran S, Thabah MM, Azharudeen M, Ramesh A, Bobby Z, Negi VS. Liver Abnormalities in Systemic Lupus Erythematosus: A Prospective Observational Study. Cureus. 2021 Jun 16;13(6):e15691. doi: 10.7759/cureus.15691. PMID: 34277279; PMCID: PMC8284411. 
2. Miller MH, Urowitz MB, Gladman DD, Blendis LM. The liver in systemic lupus erythematosus. Q J Med. 1984 Summer;53(211):401-9. PMID: 6484120. 
3. Laasonen L, Gripenberg M, Leskinen R, Skrifvars B, Edgren J. A subset of systemic lupus erythematosus with progressive cystic bone lesions. Ann Rheum Dis. 1990 Feb;49(2):118-20. doi: 10.1136/ard.49.2.118. PMID: 2317113; PMCID: PMC1003991. 
4. Harvey AM, Shulman LE, Tumulty, Conley CL, Schoenrich EH. Systemic lupus erythematosus: Review of the literature and clinical analysis of 138 cases. Medicine (Baltimore) 1954;33(4):291–437. 
5. Estes D, Christian CL. The natural history of systemic lupus erythematosus by prospective analysis. Medicine (Baltimore) 1971;50(2):85–95. 

Acknowledgements:
Thank you to Ashley Urrutia, MD, MPH, Ramoncito Caleon, MD, Steven Lewis, MD, and Peter Kenny, MD.