Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease of clinical importance in the Philippines. While its actual prevalence in the Philippines is unknown due to a lack of organized cohorts and community-level screening, Filipino females in the second and third decades of life are primarily affected, commonly presenting with mucocutaneous, musculoskeletal, hematologic, and renal involvements. Several risk factors have been described as influencing the development of SLE in Filipinos, including molecular/genetic, clinical, lifestyle, and environmental factors. The diagnosis of SLE in the Philippines still relies on international standards, such as the Systemic Lupus International Collaborating Clinics 2012 and the European League Against Rheumatism/American College of Rheumatology 2019 classification criteria. Similarly, the lack of published local guidelines for SLE requires Filipino practitioners to rely on international management guidelines, which now include the Asia-Pacific League of Associations for Rheumatology recommendations for the Asia-Pacific region, with management goals including the achievement of a low disease activity state, the prevention of organ damage, the prevention of flares, and the promotion of quality of life. Structural barriers still impede comprehensive lupus care in the country, manifesting as poor access to essential drugs and rheumatologists, low capacity for community-level surveillance, lacking research and guidelines in Philippine-specific nuances (i.e., common presentations and comorbidities such as tuberculosis), and education. The medical community in the country must be mobilized to ensure holistic care for SLE patients, as it is one of the most important rheumatologic conditions in the Philippines.

This is a rare case of primary pachydermoperiostosis (PDP). A 28-year-old Filipino male presented with a lifelong history of enlarged hands and feet. He eventually developed symmetrical swelling of the ankles and knees associated with leg heaviness and knee pain with difficulty with ambulation, hence consult. His eldest brother also had the same “elephant-like” extremities. He had cutis vertices gyrata with a thickened corrugated hair pattern, deep lines on the forehead, deepened nasolabial folds, enlarged extremities especially distally, with coarse, thick skin, and prominent clubbing. The nails were convex “watch crystal-like.” The wrists, knees, and ankles were tender and enlarged, with massive effusion of the knees. All joints were devoid of warmth and erythema. Skeletal survey favored hypertrophic osteoarthropathy over acromegaly, with periosteal thickening of the metaphysis and digital clubbing. The filarial smear was negative for blood parasites. Skin biopsy showed keratoderma. Synovial fluid was non-inflammatory while arthroscopic synovial biopsy showed chronic inflammation eosinophilic amorphous tissue. Electrocardiogram, echocardiogram, thyroid function tests, complete blood count, peripheral blood smear, serum chemistries, coagulation tests, urinalysis, urine electrolytes, fecalysis, and chest CT scan were unremarkable. Whole abdomen ultrasound revealed the liver parenchymal disease. Hepatitis profile revealed chronic infection with hepatitis B, with low infectivity. The three major criteria for PDP (pachydermia, periostitis, and digital clubbing) were fulfilled. Possible secondary causes were either excluded or were non-contributory. He was started on analgesics and anti-inflammatory medicines. Repeated arthrocenteses drained liters of synovial fluid per knee, and along with intra-articular steroid injections and compressive bandages, temporarily relieved his bilateral knee pain. He was referred to rehabilitation to maximize his range of motion and to address body image issues. The patient remains on regular follow-up for periodic arthrocentesis. The option of anti-VEGF treatment and arthrotomy was explored as possibilities but were not deemed practical. PDP is a rare genodermatosis. Life span is not affected but the quality of life is dismal without supportive management, as there is no known cure. A multidisciplinary team composed of a rheumatologist, dermatologist, orthopedic surgeon, plastic surgeon, rehabilitation physician, and a psychiatrist should be available to assist in the needs of these patients.
Osteoarthropathy, Primary Hypertrophic