2025 – PAGE 424 – RHEUMATOLOGY

NON-ARTHRITIC CONDITIONS

(DOUBLE TAKE) DERMATOMYOSITIS

Dermatomyositis results in a heliotropic, violaceous rash in malar area. Gottron’s Papules (erythematous, shiny, pruritic papules over the metacarpals) may be present. Patients will have proximal weakness and possible telangiectasias near the nail folds. Diagnose with a MUSCLE BIOPSY. The CK LEVEL WILL BE HIGH. These patients can also get calcinosis cutis/calciphylaxis.

PEARL/REMINDER: Duchenne Muscular Dystrophy also has elevated CK levels.

IMAGE: www.pbrlinks.com/DERMATOMYOSITIS1
IMAGE: www.pbrlinks.com/DERMATOMYOSITIS2
IMAGE: (calcinosis cutis) www.pbrlinks.com/DERMATOMYOSITIS3

IMMUNOGLOBULIN A VASCULITIS (FORMERLY KNOWN AS HENOCH-SCHONLEIN PURPURA) 

Immunoglobulin A vasculitis (AKA IgA vasculitis or IgAV) is a vasculitis that can involve multiple systems, including the skin, joints, GI tract, and kidneys. Classic findings include early pink, blanching, flat or urticarial lesions that later become PALPABLE and TENDER purpura. These are most often found at the lower extremities and buttocks but may be elsewhere. There may also be periarticular joint involvement (soft tissue only) at the knees or ankles, and a faint rash. Patients may initially present with colicky abdominal pain ± blood in stool ± intussusception ± gallbladder hydrops. Skin findings are impressive, but the labs show a normal platelet count. Urinalysis will likely show hematuria ± proteinuria, which can range from mild to in the nephrotic range (order a spot protein-to-creatinine ratio). This may be diagnosed on clinical findings. Complement levels are typically normal. A biopsy may be obtained if there is doubt about the diagnosis. Biopsy would show IgA, IgG, and C3 deposits. The disease often resolves without intervention, and the use of medications is debatable, meaning it’s unlikely to be tested. For the boards, give NSAIDS for joint pain in the absence of renal disease, and for severe symptoms (can’t eat) or for a hospitalized patient, give steroids with or without other therapies (i.e., cyclophosphamide, azathioprine, plasmapheresis, IVIG, etc.).

IMAGE: www.pbrlinks.com/HSP1
IMAGE: www.pbrlinks.com/HSP2

SARCOIDOSIS

Sarcoidosis presents with HILAR ADENOPATHY + multisystem complaints and findings. Patients can present with fatigue, exercise intolerance, weight loss, or a chronic cough. Also look for any evidence of problems with the eyes, heart, or kidneys. Imaging will show the classic hilar lymphadenopathy and possibly peribronchial infiltrates. If a lesion were to be biopsied, it would show the classic noncaseating granulomas.

SJOGREN SYNDROME (AKA SJOGREN’S SYNDROME)

Sjogren syndrome (AKA Sjogren’s syndrome) results in dry eyes, dry mouth, parotiditis, and possibly lab findings consistent with a renal tubular acidosis (RTA). May also have arthralgias, photophobia, and elevated ESR/CRP. The disease is caused by lymphocytic infiltration of exocrine glands. For a “first step,” start with a Schirmer’s test to look for dry eyes (a piece of paper is placed in the eye and minimal tear absorption is noted). To confirm diagnosis, biopsy the lip or a salivary gland to look for lymphocytic infiltration.