MTB Rheuma Flashcards
Felty syndrome:
Caplan syndrome:
- RA • Splenomegaly • Neutropenia
* RA • Pneumoconiosis • Lung nodules
The most common risk factors of CPPD are
hemochromatosis and hyperparathyroidism.
JRA
Fever in young
Features of JRA rash:
• Often only with fever spikes • “Salmon” colored • On chest and abdomen
Other features of JRA:
• Splenomegaly • Pericardial effusion • Mild joint symptoms
SLE presentation
Skin: Four of the manifestations of SLE are of the skin:
1. Malar rash 2. Discoid rash 3. Photosensitivity 4. Oral ulcers
Joint: Arthritis is present in 90% of those with SLE and is often the first symp-tom that brings patients to seek medical attention. SLE gives joint pain without deformation or erosion. That is why the x-ray is normal.
Serositis: Inflammation of the pleura and pericardium gives chest pain poten-tially with both pericardia! and pleural effusion.
Renal: Any degree of abnormality can occur, from mild proteinuria to end-stage renal disease requiring dialysis. The most common glomerulonephritis is membranous. Red cell casts and hematuria occur.
Neurologic: Symptoms include psychosis, seizures, or stroke from vasculitis.
Hematologic: Hemolytic anemia is part of the diagnostic criteria, but the ane-mia of chronic disease is more commonly found. Lymphopenia, leukopenia, and thromobocytopenia are also seen.
Immunologic (laboratory) abnormalities: Criteria include positive ANA, or any one of the following:
• Anti-double-stranded DNA • Anti-Sm • False positive test for syphilis • Positive LE cell preparation
Additional findings:
• Mesenteric vasculitis • Raynaud phenomenon • Antlphosphollpld syndromes
SLE diagnostic lab
ANA anti DS DNA anti sm Decreased complements Anti SSA/SSB
In an acute lupus flare,
complement levels drop and anti-DS DNA levels rise.
APL Dx
Mixing test
the aPTT will remain elevated.
Russell viper venom test (RVVT).
The RVVT is prolonged with APL antibodies and does not cor-rect on mixing with normal plasma.
Scleroderma CREST syndrome
CREST syndrome (Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, Telangiectasia).
Scleroderma presentation
Presentation
Raynaud syndrome: increased vascular reactivity of the fingers beginning with pain and pallor (white) or cyanosis (blue) followed by reactive hyperemia (red). Raynaud is precipitated by cold and emotional stress. Some cases lead to ulceration and gangrene.
Skin manifestations: fibrosis of the hands, face, neck, and extremities; telan-giectasia and abnormalities of pigmentation occur
Gastrointestinal: esophageal dysmotility with GERD, large-mouthed diver-ticuli of small and large bowel
Renal: sudden hypertensive crisis
Lung: fibrosis leading to restrictive lung disease and pulmonary hypertension
Cardiac: myocardial fibrosis, pericarditis, and heart block; lung disease gives right ventricular hypertrophy
Scleroderma diagnosis
Diagnostic Tests
ANA: positive in 85% to 90%, but nonspecific
ESR: usually normal
SCL-70: the most specific test is the SCL-70 (anti-topoisomerase), but present in only 30% of those with diffuse disease and 20% of those with limited disease
Anticentromere: present in half of those with CREST syndrome
Scleroderma treatment
Treatment
Methotrexate slows the underlying disease process of limited scleroderma. Penicillamine is not effective.
Renal crisis: ACE inhibitors (use even if the creatinine is elevated)
Esophageal dysmotility: PPis for GERD
Raynaud: calcium channel blockers
Pulmonary fibrosis: Cyclophosphamide improves dyspnea and PFTs.
Pulmonary hypertension: • Bosentan ambrisentan (endothelin antagonist) • Sildenafil • Prostacyclin analogs: iloprost, treprostinil, epoprostenol
Dermatomyositis presents with:
Proximal muscle weakness
They do not affect facial or ocular muscles as occurs in myasthenia gravis
• Malar involvement • Shawl sign: erythema of the face, neck, shoulders, upper chest, and back • Heliotrope rash: edema and purplish discoloration of the eyelids • Gottron papules: scaly patches over the back of the hands, particularly the PIP and MCP joints
Shawl sign:
Heliotrope rash:
Gottron papules:
erythema of the face, neck, shoulders, upper chest, and back
edema and purplish discoloration of the eyelid
scaly patches over the back of the hands, particularly the PIP and MCP joints
Sjogren s Dx
Schirmer test
Biopsy
All vasculitides (e.g., polyarteritis nodosa, Wegener granulomatosis, Churg-Strauss, giant cell) give:
• Fever • Malaise/fatigue • Weight loss • Arthralgia/myalgia